Thus, RMS is a common childhood malignancy with a myriad of presentations, treatments and outcomes depending on the primary site and stage. The outlook for children with these tumors has steadily improved with the multidisciplinary approach exemplified by the Intergroup Rhabdomyosarcoma Studies. The surgeon plays a pivotal role on this multidisciplinary team in the initial tumor staging and diagnosis and in complete initial resections, when judicious, or in delayed secondary resections after chemotherapy and radiotherapy. Operations on these tumors should be performed by surgeons who possess a full knowledge of the biology and treatment options of this complex tumor.