The case was a 77-year-old male with swelling of his right leg. Physical examination revealed an ill-defined mass at RLQ. Computed tomography (CT) and 3 dimensional CT showed an 8-cm tumor on the IVC, partially replacing iliac vessels and invading the psoas muscle. A diagnosis of malignant fibrohistiocytoma was made by pathological examination of biopsied specimens at exploratory laparotomy. Five courses of combination chemotherapy of ifosfamide (IFM) and doxorubicin (DXR) resulted in PR. Edema of the lower leg and hydronephrosis were both alleviated. Another 5 courses of chemotherapy with epirubicin and IFM were added. PR lasted 2 years, though the patient succumbed to the disease in 2 years and 8 months.