Seven cases of malignant inverting papillomas are presented (6 men and 1 woman) with ages ranging from 31 to 72 years (mean age: 58 years); the minimum follow up was 37 months. The diagnosis was based on the histopathologic analysis of biopsy in five patients. In two patients the diagnosis was confirmed by the pathological analysis of the specimen. Treatment was always surgical: lateral rhinotomy and total ethmoidectomy. Neoadjuvant chemotherapy was given to four patients. Post operative radiation therapy was delivered in five patients. Five patients on seven died: local recurrence and systemic metastases: 3; systemic metastases: 1; second primary: 1. Reports from the literature are discussed. The authors emphasize the treatment modalities based on complete tumor removal through lateral rhinotomy.