Abstract: Objectives To investigate the clinical diagnosis, treatment and prognosis of primary adult renal sarcoma. Methods 1654 cases of adult renal tumors were treated from 1985 to 2009, 17 cases of adult primary renal sarcoma in all of the tumors were analyzed retrospectively. Results The first symptom of 10 cases in all renal sarcomas was lumbar and abdominal mass. All 17 patients accepted radical nephrorectomy, with the pathological diagnosis of leiomyosarcoma (7 cases), rhabdomyosarcoma (2 cases), malignant fibrous histocytoma (2 cases), low-differentiated sarcoma (2 cases), and liposarcoma, fibrosarcoma, embryonic sarcoma and leiomyosarcoma coexist with chromophobe renal cell carcinoma for each one. One with tumor thrombosis in inferior vena cava died on table for pulmonary artery thrombosis. 15 cases were regularly followed up; one still lives without recurrence for 9 months, the other 14 cases died in 5 to 60 months with the average survival duration of 18 months. Average survival of leiomyosarcoma group was 28(11~60) months, and 2 case of malignant fibrous histocytoma died in 4 and 8 months respectively. Conclusion The primary renal sarcoma has the common clinical symptom similar with advanced renal cell carcinoma, and poor prognosis. Stage of tumor, pathologic type and no post-operational remained tumor are the major prognostic factors.