Congenital malformations of the nervous system
53 codes in this section
About Section Q00-Q07
Section Q00-Q07 (Q00-Q07) covers congenital malformations of the nervous system. It sits within the Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders chapter (Q00-QA0). This section contains 53 ICD-10-CM codes, 45 billable diagnoses you can submit on a claim, and 8 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 Q00-Q07
Grouped by 3-character root code. Click any code for full detail.
| Q00.0 | Anencephaly | Billable |
| Q00.1 | Craniorachischisis | Billable |
| Q00.2 | Iniencephaly | Billable |
| Q01.0 | Frontal encephalocele | Billable |
| Q01.1 | Nasofrontal encephalocele | Billable |
| Q01.2 | Occipital encephalocele | Billable |
| Q01.8 | Encephalocele of other sites | Billable |
| Q01.9 | Encephalocele, unspecified | Billable |
| Q03.0 | Malformations of aqueduct of Sylvius | Billable |
| Q03.1 | Atresia of foramina of Magendie and Luschka | Billable |
| Q03.8 | Other congenital hydrocephalus | Billable |
| Q03.9 | Congenital hydrocephalus, unspecified | Billable |
| Q04.0 | Congenital malformations of corpus callosum | Billable |
| Q04.1 | Arhinencephaly | Billable |
| Q04.2 | Holoprosencephaly | Billable |
| Q04.3 | Other reduction deformities of brain | Billable |
| Q04.4 | Septo-optic dysplasia of brain | Billable |
| Q04.5 | Megalencephaly | Billable |
| Q04.6 | Congenital cerebral cysts | Billable |
| Q04.8 | Other specified congenital malformations of brain | Billable |
| Q04.9 | Congenital malformation of brain, unspecified | Billable |
| Q05.0 | Cervical spina bifida with hydrocephalus | Billable |
| Q05.1 | Thoracic spina bifida with hydrocephalus | Billable |
| Q05.2 | Lumbar spina bifida with hydrocephalus | Billable |
| Q05.3 | Sacral spina bifida with hydrocephalus | Billable |
| Q05.4 | Unspecified spina bifida with hydrocephalus | Billable |
| Q05.5 | Cervical spina bifida without hydrocephalus | Billable |
| Q05.6 | Thoracic spina bifida without hydrocephalus | Billable |
| Q05.7 | Lumbar spina bifida without hydrocephalus | Billable |
| Q05.8 | Sacral spina bifida without hydrocephalus | Billable |
| Q05.9 | Spina bifida, unspecified | Billable |
| Q06.0 | Amyelia | Billable |
| Q06.1 | Hypoplasia and dysplasia of spinal cord | Billable |
| Q06.2 | Diastematomyelia | Billable |
| Q06.3 | Other congenital cauda equina malformations | Billable |
| Q06.4 | Hydromyelia | Billable |
| Q06.8 | Other specified congenital malformations of spinal cord | Billable |
| Q06.9 | Congenital malformation of spinal cord, unspecified | Billable |
| Q07.0 | Arnold-Chiari syndrome | Header |
| Q07.00 | Arnold-Chiari syndrome without spina bifida or hydrocephalus | Billable |
| Q07.01 | Arnold-Chiari syndrome with spina bifida | Billable |
| Q07.02 | Arnold-Chiari syndrome with hydrocephalus | Billable |
| Q07.03 | Arnold-Chiari syndrome with spina bifida and hydrocephalus | Billable |
| Q07.8 | Other specified congenital malformations of nervous system | Billable |
| Q07.9 | Congenital malformation of nervous system, 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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