This study evaluates limb-saving surgery (LSS) combined with intraoperative radiotherapy (IORT) and external beam radiotherapy (EBRT) for extremity sarcoma. Prospectively gathered data was analyzed for 50 patients. Advanced stages (UICC IIB-IIIB) predominated (80%) and 50% of the tumors were recurrent. Early local morbidity amounted to 32%, including wound infection (6), skin necrosis (3), hematoma (3) and others (4). Late morbidity of 10% consisted of a fracture, a fibrosis, two chronic lymphedemas and a neuropathy. One patient died (pulmonary embolism). Local control was obtained in 91% with an overall 3-year survival rate of 81% in cases treated with curative intent (follow-up 29 months). No survival benefit was found for patients with microscopically clear margins; however, local failure occurred less frequently (p = 0.02). Our results support the use of LSS with IORT and EBRT for extremity sarcoma and emphasize the importance of the surgical margin.