Other congenital malformations
98 codes in this section
About Section Q80-Q89
Section Q80-Q89 (Q80-Q89) covers other congenital malformations. It sits within the Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders chapter (Q00-QA0). This section contains 98 ICD-10-CM codes, 81 billable diagnoses you can submit on a claim, and 17 non-billable header codes used to organize the hierarchy.
Codes are organized below by 3-character root. Click the root to see all subcodes underneath it, or click any individual code for full coverage data, payer policies, and linked CPT procedures.
Codes in Q80-Q89
Grouped by 3-character root code. Click any code for full detail.
| Q80.0 | Ichthyosis vulgaris | Billable |
| Q80.1 | X-linked ichthyosis | Billable |
| Q80.2 | Lamellar ichthyosis | Billable |
| Q80.3 | Congenital bullous ichthyosiform erythroderma | Billable |
| Q80.4 | Harlequin fetus | Billable |
| Q80.8 | Other congenital ichthyosis | Billable |
| Q80.9 | Congenital ichthyosis, unspecified | Billable |
| Q81.0 | Epidermolysis bullosa simplex | Billable |
| Q81.1 | Epidermolysis bullosa letalis | Billable |
| Q81.2 | Epidermolysis bullosa dystrophica | Billable |
| Q81.8 | Other epidermolysis bullosa | Billable |
| Q81.9 | Epidermolysis bullosa, unspecified | Billable |
| Q82.0 | Hereditary lymphedema | Billable |
| Q82.1 | Xeroderma pigmentosum | Billable |
| Q82.2 | Congenital cutaneous mastocytosis | Billable |
| Q82.3 | Incontinentia pigmenti | Billable |
| Q82.4 | Ectodermal dysplasia (anhidrotic) | Billable |
| Q82.5 | Congenital non-neoplastic nevus | Billable |
| Q82.6 | Congenital sacral dimple | Billable |
| Q82.8 | Other specified congenital malformations of skin | Billable |
| Q82.9 | Congenital malformation of skin, unspecified | Billable |
| Q83.0 | Congenital absence of breast with absent nipple | Billable |
| Q83.1 | Accessory breast | Billable |
| Q83.2 | Absent nipple | Billable |
| Q83.3 | Accessory nipple | Billable |
| Q83.8 | Other congenital malformations of breast | Billable |
| Q83.9 | Congenital malformation of breast, unspecified | Billable |
| Q84.0 | Congenital alopecia | Billable |
| Q84.1 | Congenital morphological disturbances of hair, not elsewhere classified | Billable |
| Q84.2 | Other congenital malformations of hair | Billable |
| Q84.3 | Anonychia | Billable |
| Q84.4 | Congenital leukonychia | Billable |
| Q84.5 | Enlarged and hypertrophic nails | Billable |
| Q84.6 | Other congenital malformations of nails | Billable |
| Q84.8 | Other specified congenital malformations of integument | Billable |
| Q84.9 | Congenital malformation of integument, unspecified | Billable |
| Q85.0 | Neurofibromatosis (nonmalignant) | Header |
| Q85.00 | Neurofibromatosis, unspecified | Billable |
| Q85.01 | Neurofibromatosis, type 1 | Billable |
| Q85.02 | Neurofibromatosis, type 2 | Billable |
| Q85.03 | Schwannomatosis | Billable |
| Q85.09 | Other neurofibromatosis | Billable |
| Q85.1 | Tuberous sclerosis | Billable |
| Q85.8 | Other phakomatoses, not elsewhere classified | Header |
| Q85.81 | PTEN hamartoma tumor syndrome | Billable |
| Q85.82 | Other Cowden syndrome | Billable |
| Q85.83 | Von Hippel-Lindau syndrome | Billable |
| Q85.89 | Other phakomatoses, not elsewhere classified | Billable |
| Q85.9 | Phakomatosis, unspecified | Billable |
| Q86.0 | Fetal alcohol syndrome (dysmorphic) | Billable |
| Q86.1 | Fetal hydantoin syndrome | Billable |
| Q86.2 | Dysmorphism due to warfarin | Billable |
| Q86.8 | Other congenital malformation syndromes due to known exogenous causes | Billable |
| Q87.0 | Congenital malformation syndromes predominantly affecting facial appearance | Billable |
| Q87.1 | Congenital malformation syndromes predominantly associated with short stature | Header |
| Q87.11 | Prader-Willi syndrome | Billable |
| Q87.19 | Other congenital malformation syndromes predominantly associated with short stature | Billable |
| Q87.2 | Congenital malformation syndromes predominantly involving limbs | Billable |
| Q87.3 | Congenital malformation syndromes involving early overgrowth | Billable |
| Q87.4 | Marfan syndrome | Header |
| Q87.40 | Marfan syndrome, unspecified | Billable |
| Q87.41 | Marfan syndrome with cardiovascular manifestations | Header |
| Q87.410 | Marfan syndrome with aortic dilation | Billable |
| Q87.418 | Marfan syndrome with other cardiovascular manifestations | Billable |
| Q87.42 | Marfan syndrome with ocular manifestations | Billable |
| Q87.43 | Marfan syndrome with skeletal manifestation | Billable |
| Q87.5 | Other congenital malformation syndromes with other skeletal changes | Billable |
| Q87.8 | Other specified congenital malformation syndromes, not elsewhere classified | Header |
| Q87.81 | Alport syndrome | Billable |
| Q87.82 | Arterial tortuosity syndrome | Billable |
| Q87.83 | Bardet-Biedl syndrome | Billable |
| Q87.84 | Laurence-Moon syndrome | Billable |
| Q87.85 | MED13L syndrome | Billable |
| Q87.86 | Kleefstra syndrome | Billable |
| Q87.87 | Hao-Fountain Syndrome | Billable |
| Q87.88 | CTNNB1 syndrome | Billable |
| Q87.89 | Other specified congenital malformation syndromes, not elsewhere classified | Billable |
| Q89.0 | Congenital absence and malformations of spleen | Header |
| Q89.01 | Asplenia (congenital) | Billable |
| Q89.09 | Congenital malformations of spleen | Billable |
| Q89.1 | Congenital malformations of adrenal gland | Billable |
| Q89.2 | Congenital malformations of other endocrine glands | Billable |
| Q89.3 | Situs inversus | Billable |
| Q89.4 | Conjoined twins | Billable |
| Q89.7 | Multiple congenital malformations, not elsewhere classified | Billable |
| Q89.8 | Other specified congenital malformations | Header |
| Q89.81 | Kabuki syndrome | Billable |
| Q89.89 | Other specified congenital malformations | Billable |
| Q89.9 | Congenital malformation, unspecified | Billable |
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Reviewed by the PayerReady Medical Coding Team
Verified against the CMS 2026 code set on May 31, 2026.
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