From 1970 to 1987, 34 patients younger than 22 years of age with extremity rhabdomyosarcoma were treated at the Memorial Sloan-Kettering Cancer Center (MSKCC). All patients were treated according to protocols consisting of surgery, radiotherapy, and multiple drug chemotherapy. Sixteen of 34 patients survived, and 14 continue to be disease-free; the 5-year survival rate was 44%. A retrospective univariate analysis of data according to the TNM staging system was undertaken. Tumor invasion, regional nodal involvement, distant metastases, and alveolar histologic condition each had a significant impact on survival. However, in multivariate analysis, the stage of disease at diagnosis was the most important predictor of survival outcome.