Congenital cholesteatoma is a rare entity. It arises from aberrant epithelial remnants left at the time of closure of the neural groove, between the 3rd and 5th week of embryonic life. Otoscopy typically shows a pearly white mass, medial to the intact tympanic membrane, primarily in the anterosuperior quadrant, without a preceding history of otorrhoea, tympanic membrane perforation or surgery. It can present at any age from birth to early adulthood. The most common presenting symptom is hearing loss or facial nerve palsy. We present a non-classical presentation of congenital cholesteatoma with loss of taste sensation and numbness on the anterior two-thirds of the tongue associated with a bulging posterosuperior quadrant of the ipsilateral tympanic membrane in a 52-year-old male. After investigation, the patient underwent surgical management resulting in improvement of his symptoms.