Malignant fibrous histiocytoma (MFH) with fibroblastic and histiocytic characteristics, an extremely high grade soft tissue invasive sarcoma, occurs most commonly in 50-70 year old males, especially in the extremities and more rarely, the head and neck MFH metastasizes in 20% of cases, more commonly to the lung (90%), the head and neck (very rare) and the nasal septum (extremely rare). A 52-year-old male was diagnosed with MFH of the left popliteal fossa by an outside orthopedic clinic and accepted resection of the primary site, chemotherapy and radiotherapy, before visiting our hospital in September 2004. The tumor recurred at the same site one year later and metastasizing to the lung and brain. Admitted to our hospital for chemotherapy, the patient complained mainly of nasal congestion. After consulting an ENT doctor, a 1×1 cm^2 gray tumor on the left side of the nasal septum with blood-streaked discharge was evinced by nasopharyngoscope. The histiocytoma was removed surgically and pathologically proven. We herein discuss the diagnosis and treatment of this case of MFH and review literature relevant to histiocytoma.