Huge mastoid congenital cholesteatoma in a 52-year-old patient.

Congenital cholesteatoma may arise in various locations within the temporal bone. The rarest site of origin is the mastoid process. We report an unusual case of a 52-year-old man with mastoid congenital cholesteatoma that manifested as a persistent ear discharge. The preoperative suspicion was based on the imaging findings and the patient's history. A simple mastoidectomy was conducted and the cholesteatoma was completely removed while using facial nerve monitoring. Although rare, mastoid congenital cholesteatoma can be considered as an alternative in the differential diagnosis of persistent otorrhea.