Congenital cholesteatoma is a rare entity. It may originate at various sites in the temporal bone, for example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or in the external auditory canal. The least common site being the mastoid process. Most common presentation is a retrotympanic pearly white mass with no previous history of ear discharge, perforation or any ear surgery. It can lead to various complications, both intracranial and extracranial, some of which may be life threatening. Bezold's abscess is an extracranial complication which is usually seen in children following acute otitis media with mastoiditis. Here we present a rare case of a 60 year old patient with congenital cholesteatoma complicating to Bezold's abscess. After necessary investigations patient underwent surgery for complete removal of cholesteatoma and the abscess drainage.