Inverted papilloma is an uncommon benign epithelial tumor that typically affects the nasal cavity and originates from the lateral sinonasal wall. Inverted papilloma occurring in the middle ear is an even more rare and aggressive condition. We describe a case of a 76-year-old man who was treated for isolated sinonasal inverted papilloma and after 20 months he was found to have a recurrence in the nasal cavity with middle ear involvement and malignant changes consistent with nonkeratinizing squamous cell carcinoma arising from inverted papilloma. Several surgical procedures were required to remove the tumor along with radiation therapy postoperatively.