Epidermoid tumors are rare, benign congenital lesions which typically present between the third and fifth decades of life. They are thought to originate from ectodermal cells misplaced during neural tube formation and separation. While epidermoids may present anywhere in the cranial vault, they are characteristically located intradurally and in a paramedian position within the cerebellopontine angle and parasellar regions. Although imaging results may vary depending upon cystic content, CT scanning generally reveals a well-circumscribed, nonenhancing, lobulated, hypodense mass. They are hypointense on T1-weighted MRI, and hyperintense on T2-weighted MRI, diffusion-weighted imaging and fluid-attenuated inversion recovery sequences. The use of appropriate neuroimaging should be utilized to differentiate epidermoids from other intracranial lesions. While gross total resection of these tumors is the definitive treatment to prevent recurrence and aseptic meningitis, a subtotal resection may be necessary to preserve neurological function.

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