Congenital cholesteatoma is a cyst of keratinizing squamous cell epithelium in the setting of an intact tympanic membrane, in a patient without a history of otorrhea, tympanic membrane perforation, or otologic surgery. The most common presentation of a congenital cholesteatoma is that of an asymptomatic pearly white mass in the anterosuperior quadrant of the tympanic cavity. The etiology of congenital cholesteatoma has been debated at length, with the leading theory being the epithelial rest theory. Treatment for congenital cholesteatoma is surgical, with advances in endoscopic ear surgery allowing for improved intraoperative visualization and postoperative lowered recidivism rates.