The term cholesteatoma refers to "chole": cholesterol, "steat": fat and "oma,": "tumor". This tumor has been reported to be the most common in the middle ear. The occurrence of such a tumor in the maxillary sinus is deemed to be very rare and hardly 4 cases were reported in India and 26 cases described worldwide. This case report intends to discuss the uniqueness and indolent nature of this lesion in terms of histopathology and radiography.