Huge Arachnoid Cyst of the Posterior Fossa with Cerebellar Tentorium Dysplasia Associated with Juvenile Polyposis

We report an infant with a huge arachnoid cyst of the posterior fossa with dysplasia of the cerebellar tentorium and meningeal sinus and associated juvenile polyposis. Neuroimaging studies disclosed a huge median cystic lesion extending posterosuperiorly over the cerebellum. The cerebellar tentorium was raised to the parietal area; the vermis was normoplastic. Cystography showed no direct communication with the 4th ventricle or subarachnoid space. We discuss the differential diagnosis of median cysts of the posterior fossa and the association of juvenile polyposis.