Rhabdomyosarcoma (RMS) is a tumor primarily of mesenchymal origin that comprises approximately half of all pediatric sarcomas, which make up 7% of all pediatric tumors. The focus of this manuscript will include recent changes in surgical and local control therapy of RMS. We will discuss surgical therapy principles including improved outcomes associated with complete surgical resection of the primary tumor either prior to the initiation of chemotherapy or after induction of chemotherapy, and in the setting of metastatic disease. We will also discuss the increasing utilization of core needle biopsy for diagnosis and the use of sentinel node biopsy techniques to determine the presence of nodal disease. There have been refinements regarding optimal prognostic factors including the adoption of fusion status over histology, as well as updated risk stratification schemas to assure optimal therapy. Since this manuscript is an international collaboration, part of the discussion will also focus on comparisons between North American and European surgical paradigms.