Inverted papilloma of the nasal cavity and paranasal sinuses is uncommon, but malignant lymphoma of the nasal cavity and paranasal sinus is more rare. A case of co-existent inverted papilloma and malign-nant lymphoma of the nasal cavity and paranasal sinus was presented. The patient was treated by left medial maxillectomy and two courses of chemotherapy postoper-atively. The result was successful and there has been no local recurrence observed till now. The connection between inverted papilloma and carcinoma is well estab-lished. Howerever, there has been no report in the literature about co-existence of inverted papilloma and malignant lymphoma. The presented case was well discussed.