Aicardi syndrome is an X-linked dominant disorder primarily defined by the triad of corpus callosum agenesis, infantile spasms and a pathognomonic lacunar chorioretinopathy. Papillomas of the choroid plexus have been reported in affected patients. We report an Aicardi syndrome patient who had three separate choroid plexus papillomas and associated hydrocephalus. A dizygotic twin was unaffected. Staged resection of the tumors was safely accomplished, with improvement in seizure control. Imaging between procedures revealed rapid tumor growth.

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