Therapy in metastatic Ewing's sarcoma is reviewed using the methodology recommended by the guidelines project of the Federation of French Cancer Centres (FNCLCC) Standards, Options and Recommendation (SOR) Group. Twelve articles relating to conventional dose therapy and seven articles related to high-dose therapy were judged suitable for detailed appraisal. Rates of complete response (CR) at metastatic sites and local control were high using combinations of vincristine, actinomycin, cyclophosphamide and doxorubicin with radiation or surgery. With more recent regimens, including increased doses of alkylating agents and anthracyclines the relapse-free survival has increased from < 15 to 20-30%. 'Megatherapy' regimens with haematopoietic stem cell rescue are tolerable in this patient group, but to date there is little evidence of any benefit. It appears that patients with isolated lung metastases do significantly better (approximately 40% EFS) than those presenting with combined sites such as bone, bone marrow and lung. The use of lung irradiation in children with lung metastases is associated with a reduced incidence of subsequent lung recurrence and a consistently better overall relapse-free survival (RFS).