AbstractCongenital middle ear encephalocele (CMEE) is a rare entity, previously reported in only 29 patients. It might originate from dehiscence of the tegmen tympani or antri or the bony plate of the posterior fossa. The common presenting symptoms are spontaneous cerebrospinal fluid (CSF) otorrhea and/or rhinor‐rhea, persistent “serous otitis media,” conductive hearing loss, and, occasionally, recurrent meningitis. We report a case of CMEE initially seen with progressive facial paresis, review the previously reported cases of CMEE, and discuss the pathogenesis and surgical manag