Inverted papilloma (IP) of the middle ear as a primary lesion or as an extension of a sinonasal papilloma, is extremely rare. Only 23 cases of primary inverted papilloma of the middle ear have been reported in the literature. They are locally aggressive tumours, with a high rate of recurrence and associated malignancy. We present a rare case of a 59-year-old man presenting with unilateral otorrhoea, tinnitus, and hearing loss. Otoscopy revealed posterior perforation filled by irregular budding neoformation. The temporal CT scan showed tissue filling the tympanic cavity as well as the mastoid antrum without bone lysis. The patient underwent limited tympanoplasty. An intraoperative biopsy of polypoid tissue filling the tymapanic cavity was made and histopathology showed an IP. A recurrence occurred 4 months after surgery. We performed open tympanoplasty allowing complete resection of the lesions, with no recurrence after a follow-up of 30 months.