OBJECTIVE To review an institutional experience with radiotherapy (RT) in patients with metastatic Ewing sarcoma. METHODS Thirty patients with metastatic Ewing sarcoma were considered. Twenty-nine received multiagent chemotherapy, whereas 22 had local therapy, which included RT in 14, surgery in 5, and surgery followed by RT in 3. RESULTS The 5-year overall survival rate was 22.1%. On multivariate analysis, presence of uncommon sites of metastasis (brain, liver, spleen) (P<0.0001) and use of local therapy to the primary site (P<0.001) were adverse factors for survival. Local control was not achieved in the 8 patients receiving only chemotherapy. All long-term survivors had local therapy including RT to metastatic bony sites and whole-lung irradiation for pulmonary metastasis. CONCLUSIONS The presence of uncommon sites of metastasis confers a worse prognosis. Aggressive primary treatment including RT to metastatic sites should be considered in these patients.