Meningoencephaloceles of the temporal bone are rare. Although most often seen following otologic surgery or trauma, congenital meningoencephaloceles can exist. The clinical presentation, diagnostic evaluation, and surgical management of three patients with congenital meningoencephalocele are presented. Two of the three patients presented to our institution with recurrent episodes of meningitis; one presented with partial complex seizures. Diagnostic evaluation included temporal bone computed tomography with magnetic resonance imaging. In two patients, defects were imaged following high-pressure subarachnoid cisternography with computed tomography. All three patients were found to have congenital defects in the area of Meckel's cave. Early recognition of congenital meningoencephalocele is important to avoid delay of definitive surgical management and neurologic sequelae.