Abstract
Sturge weber syndrome is a neurocutaneous disease that manifests with vascular malformations involving the brain, eye and skin. It is a rare disorder that occurs with a sporadic frequency of 1:50000. The condition is characterized by facial nevus, seizures and intracranial calcifications. We present a case of sturge weber syndrome with corpus callosal agenesis; unreported in literature.An eight year old boy presented with generalized tonic clonic seizures. There was port wine stain over right upper half of the face and eyelid. CT scan of brain showed atrophy of right occipital lobe with a gyriform calcification in parasaggital region. The posterior horns of the lateral ventricles were dilated. The third ventricle was communicating with inter hemispheric fissure suggesting agenesis of corpus callosum.The angiomatosis might have caused agenesis of corpus callosum either by tissue hypoxia or by mechanical interference of the development.
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