Congenital primary cholesteatoma of external auditory canal

A 2-year-old female child is reported with congenital cholesteatoma of the right external auditory canal. We describe the clinical features, computed tomography finding and surgical treatment. Congenital cholesteatomas can occur within the temporal bone. Congenital cholesteatoma of the external auditory canal is rare. Generally, it appears in the canal floor. Treatment consists of the resolution of granulation and removal of debris.