Congenital Cholesteatoma is a rare disease which originated from the ectoderm that from the primitive notochord and embryonal cell rests. Their rests from the ectodermal structure may occur in any portion of the cranial bones. Schuknecht classified the location of congenital cholesteatoma into deep portion in the petrous bone, in the jugular fossa region and middle ear cleft. Lipkin proposed diagnostic criteria as intact tympanic membrane, no previous history of middle ear infection and embryonal cell inclusion of squamous epithelium. We recently experienced a case of huge congenital cholesteatoma in 31 year old male patient who showed normal tympanic membrane and ossicular continuity. The huge cholesteatoma was completely filled within mastoid cavity and destruction of mastoid tip forming Bezold's abscess was observed. The lateral sinus and jugular bulb were also exposed but facial nerve and lateral and posterior semicircular canal were preserved. We report this case this with review of literatures.