OBJECTIVE To analyze treatment and survival in 34 patients (28 resected) with primary or recurrent retroperitoneal sarcoma (RPS). METHODS Between July 1994 and January 2001, 34 patients (15M, 19F; mean age: 56 years, range: 25-77) were evaluated. Complete resection was defined as removal of gross tumor with histologically confirmed clear resection margins. RESULTS Twenty-eight out of 34 patients (82%) (15 were affected by primary RPS, and 13 by recurrent RPS), underwent surgical exploration. Twenty-three patients had a grossly and microscopically complete resection, (3 having a grossly incomplete resection and 2 patients with a grossly complete resection having histologically involved resection margins). Twenty-one out of 28 patients (75%) underwent removal of contiguous intra-abdominal organs. Preoperative mortality was nil, and morbidity occurred in six cases only (21%). High tumor grade results a significant variable for a worse survival in all 28 patients (100% 5 years survival for low grade vs. 0% for high grade; P = 0.0004). Amongst completely resected patients, only histologic grade and peroperative blood transfusions affected disease-free survival (P = 0.04 and P = 0.05, respectively). CONCLUSIONS An aggressive surgical approach in both primary and recurrent RPS is associated with long-term survival.