Meningoencephalic herniation into the temporal bone is a rare potentially life threatening condition. Clinical presentations include cerebrospinal fluid (CSF) otorrhea, recurrent meningitis and focal neurologic signs. However, a meningocele may mimic middle ear diseases in cases without overt CSF leakage or neurologic symptoms. Here we describe a case of meningocele herniation in the middle ear cavity with concurrent otitis media, diagnosed during operation and surgically repaired. Review of diagnostic clues and repair approaches is presented.