A case of malignant fibrous histiocytoma of the nasal septum is described in a 51 -year-old woman who has presented with nasal pain, nasal obstruction and a swelling at the base of the nose. Histological examination revealed a malignant fibrous histiocytoma. Total resection of the tumor was performed and, 26 months after operation, the patient was well and free of recurrence or metastasis. This is the first reported case of malignant fibrous histiocytoma of the nasal septum.