Rhabdomyosarcoma is the most common soft tissue tumor in childhood. During the last 30 years, the introduction of multimodal therapy has resulted in a significant improvements in survival, with a create of approximately 70% for patients with localized disease. Several trials from Collaborative pediatric groups such as the Intergroup Rhabdomyosarcoma Study Group (IRSG) have revolutionized the therapeutic methods for this sarcoma. Based on the conclusions of these studies, multimodal treatment regimens, involving surgery, chemotherapy and/or radiation, are decided by tumor staging (based on tumor primary site, tumor size, the presence or not of regional lymph node involvement and of distant metastasis), grouping (defined by the amount of residual tumor after initial surgery), and the histologic subtype of the tumor.