It is well known that inverted papilloma (IP) is sometimes associated with malignancies; however, the association of IP with verrucous carcinoma (VC) is extremely rare. We herein report a case of IP in the nasal cavity with VC in the maxillary sinus. A 73-year-old Japanese woman presented with a 3-month history of right-sided nasal obstruction and repetitive epistaxis. A biopsy of the mass was performed and the pathological result proved to be IP. Computed tomography and magnetic resonance imaging revealed a moderately enhanced soft-tissue lesion filling the right nasal cavity and a ring-enhanced expansive lesion in the maxillary sinus. Under a tentative diagnosis of IP of the nasal cavity with maxillary empyema, she underwent right medial maxillectomy via a lateral rhinotomy approach, and a histopathological diagnosis of IP in the nasal cavity with VC in the maxillary sinus was obtained. Her postoperative clinical course was uneventful. She is currently free from symptoms, and there is no evidence of recurrence 5 months after surgery. The pathology, clinical manifestation, and treatment of VC associated with IP are reviewed from literature.