Rhabdomyosarcoma (RMS) is a complex primary malignancy, comprising over half of all pediatric soft tissue sarcomas, making it the most common soft tissue sarcoma in children and adolescents. There are approximately 250–350 cases of childhood RMS in the United States each year. Like other soft tissue sarcomas, RMS arises from embryonic mesenchymal tissue, with the specific capacity to differentiate into skeletal muscle. While sarcomas in adults most commonly occur in the extremities, RMS in children may arise in any location where skeletal muscle is found. In addition, it may arise in locations where skeletal muscle is not typically found, including the bladder and bile ducts. RMS is the only tumor whose prognosis is related to the site of origin; therefore, recent staging and treatment strategies are not only based on extent of tumor but also location. Since the first case of RMS was described in 1854 by Webner, management of RMS has evolved from radical surgical excisions with poor prognosis to multimodal therapy that now often includes radiation and chemotherapy. This chapter reviews the typical presentation, workup, and surgical management of childhood RMS.