Background. Retroperitoneal sarcomas are malignant tumors with an aggressive course of disease. Cause of death is local disease in 50% and disseminated disease in only 25%. We made a retrospective analysis of surgical treatment of retroperitoneal sarcomas in order assess the effect of this treatment modality on the course of the disease.In the years between 1975 and 2000, 155 patients were surgically treated for primary retroperotneal sarcoma at the Institute of Oncology in Ljubljana. Only 81 of 155 patients received the first treatment at our Institute, while other patients had been at least once operated on elsewhere before the admission to our Institute. Of these patients, 40 required a second surgery for the residual disease and 31 for recurrence. In 23 patients, metastatic spread was found at diagnosis. Our treatment approach was aggressive. We surgically removed the recurrent sarcomas and metastases wherever accessible. Operability at the first surgical treatment performed at the Institute of Oncology was 92%. Therefore, 44 patients underwent 3 or more surgeries for sarcoma. The highest number of operations performed in one patients was 9 (2 patients). In 127 patients, the tumor block resection involved at least one additional organ (up to 6).Results. Complications were not sparse; perioperative mortality was 8.4%. The survival depended upon the metastatic spread at diagnosis, tumor grade and oncologic surgery type.Conclusions. Despite complications, only complete resection without microscopic resuduum and contamination yields a long-term survival to the patients with retroperitoneal sarcoma.