A 79-year-old man who had low grade fever and general malaise was referred to our hospital on January 27, 2004. Physical examination revealed a hard fixed painless mass, measuring 10 cm in the left abdomen. Sarcoma of retroperitoneal origin was suspected preoperatively by abdominal ultrasonogram, CT, MRI and Angiography. Extended surgical treatment including left nephrectomy was performed. The tumor was histologically diagnosed as storiform-pleomorphic malignant fibrous histiocytoma (MFH) arising from perirenal tissue. Although we recommended adjuvant therapy after the excision, the patient refused. After a follow-up period of 11 months, he developed local recurrence in the left retroperitoneal space, and external irradiation was performed. He died 21 months after the operation. To our knowledge, this is the 10th case of MFH arising from perirenal tissue in Japan.