Abstract
Congenital cholesteatoma is a disease of young. However, it also been reported in adult and also elderly. The theory of cholesteatoma formation is still debatable. Could the late presentation of congenital cholesteatoma actually acquired adult cholesteatoma? Complications are more common in congenital cholesteatoma than acquired cholesteatoma computed tomography (CT) scan is a gold standard for cholesteatoma imaging but magnetic resonance imaging (MRI) offer a better option in recurrent disease and fusion of MRI and CT scan should be considered in complicated disease. Canal wall down mastoidectomy is the treatment of choice for more extensive cholesteatoma but the risk of recurrent is depend more on extent of the disease rather than type of operations. We are reporting an adult diagnosed to have congenital cholesteatoma complicated by facial nerve palsy with normal tympanic membrane.

Key words: Congenital, Hearing loss, cholesteatoma, mastoid, facial paralysis