Short answer · Medically reviewed summary · Last updated: 2026-05-08

Agenesis of the corpus callosum (ACC) does not have a single, unique ICD-10 or ICD-9 code, but is typically classified under the code Q04.0 (Congenital malformations of the brain; Agenesis of corpus callosum) in the ICD-10 system. In the older ICD-9 system, Agenesis of the corpus callosum was generally categorized under code 742.1, which covers microcephaly and other congenital anomalies of the brain. What is the clinical classification of Agenesis of the Corpus Callosum? Agenesis of the corpus callosum is a rare congenital brain disorder where the thick band of nerve fibers connecting the two cerebral hemispheres is partially or completely absent.

2 people with Agenesis Of Corpus Callosum have shared their first-person experience on this question at DiseaseMaps.

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ICD10 code of Agenesis Of Corpus Callosum and ICD9 code

ICD-10 and ICD-9 codes for Agenesis Of Corpus Callosum, with classification details for clinicians, coders and patients.

ICD9 and ICD10 codes of Agenesis Of Corpus Callosum

Agenesis of the corpus callosum (ACC) does not have a single, unique ICD-10 or ICD-9 code, but is typically classified under the code Q04.0 (Congenital malformations of the brain; Agenesis of corpus callosum) in the ICD-10 system. In the older ICD-9 system, Agenesis of the corpus callosum was generally categorized under code 742.1, which covers microcephaly and other congenital anomalies of the brain.



What is the clinical classification of Agenesis of the Corpus Callosum?


Agenesis of the corpus callosum is a rare congenital brain disorder where the thick band of nerve fibers connecting the two cerebral hemispheres is partially or completely absent. Because Agenesis of the corpus callosum can present as an isolated finding or as part of a larger genetic syndrome, clinicians use these ICD codes primarily for administrative and billing purposes. Within our DiseaseMaps community, 304 people with Agenesis of the corpus callosum have connected to share their unique diagnostic journeys, highlighting that the clinical presentation varies widely from asymptomatic individuals to those with significant developmental challenges.



How are diagnosis and coding for Agenesis of the Corpus Callosum managed?


While the ICD-10 code Q04.0 is the standard for reporting Agenesis of the corpus callosum, physicians often append secondary codes to reflect associated conditions. Many individuals with Agenesis of the corpus callosum also experience comorbidities that require distinct coding for insurance and clinical management, including:



  • G40: Epilepsy or seizure disorders, which occur in a significant subset of patients.

  • F80-F89: Developmental disorders of speech and language.

  • Q04.3: Other specified malformations of the brain, often used when ACC is part of a complex syndrome.

  • Q87.8: Other specified congenital malformation syndromes, used when Agenesis of the corpus callosum is part of a genetic diagnosis.



Why is accurate coding important for patients with Agenesis of the Corpus Callosum?


Accurate diagnostic coding for Agenesis of the corpus callosum is essential to ensure that patients receive appropriate therapeutic support, such as physical, occupational, or speech therapy. By using the correct ICD-10 designation, healthcare providers can better track the longitudinal health outcomes of those living with Agenesis of the corpus callosum and facilitate access to specialized neurological care.



Next steps



  • Consult with a pediatric neurologist or clinical geneticist to confirm if your Agenesis of the corpus callosum diagnosis is isolated or part of a genetic syndrome.

  • Connect with the 304 members of our DiseaseMaps community to share insights on navigating medical care and insurance coding.

  • Request a copy of your clinical summary from your neurologist to ensure your medical records accurately reflect all aspects of your condition.



Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.



References



  • NIH Genetic and Rare Diseases (GARD) Information Center: Agenesis of the corpus callosum.

  • Orphanet: Rare disease database entry for Agenesis of the corpus callosum (ORPHA:1205).

  • OMIM (Online Mendelian Inheritance in Man): Corpus Callosum, Agenesis of.

  • ICD-10 Version: 2019, World Health Organization, Q04.0.

Medical disclaimer: This information does not substitute professional medical advice. Always consult your doctor before making health decisions.
Source: DiseaseMaps.org
3 answers
ICD10 code is Q04.0

Posted Sep 11, 2017 by Nickie 2500
I dont know about ICd9

Posted Oct 30, 2017 by alex 3050

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Mason Cayden Hunter-Colyer Born 11 March 2016 Diagnosis 19 weeks in utero There is also a duplication on his X chromosome that they believe is unrelated and will have no effect. At 18 months old he is a tornado on two legs with no delays, he's ...
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