Abstract: Rhabdomyosarcoma accounts for approximately 4% of cancer cases among children aged 0 to 18 years, as well as among adolescents and young adults. Moreover, rhabdomyosarcoma accounts for approximately 50% of the cases of soft-tissue sarcomas. Children afflicted with this disease who received multimodality therapy experienced a 5-year event-free survival rate of approximately 70%. The multimodality therapy entails surgical resection, the success of which is feasible without major functional/cosmetic impairment, is followed by chemotherapy. Rhabdomyosarcoma arises from various sites; thus, surgical care must be tailored to the unique aspects of each site. The aim of this article is to review the surgical therapy of rhabdomyosarcoma occurring in different parts of the body. Cosmetic and functional factors are always considered. However, with modem techniques, complete resection in patients with superficial tumors need not be inconsistent with good cosmetic and functional results, especially in several sites. For head and neck tumors, cosmetic and functional factors are always considered, although narrower resection margins (< 1 mm) are acceptable because of anatomic restrictions. The definitive surgical procedure of the extremity sites involves wide local excision with en bloc removal of a cuff of normal tissue. Most patients who present with large tumors in truncal sites have a localized disease suitable for complete resection with negative margins after preoperative therapy. Lesions occurring adjacent to the testis or spermatic cord up to the internal inguinal ring should be removed by orchiectomy with resection of the entire spermatic cord. The removal process utilizes an inguinal incision with proximal vascular control. Bladder salvage is an important goal of therapy for patients with tumors arising in the prostate and bladder. After all, the basic principle of the initial surgical treatment of children with rhabdomyosarcoma is the complete resection of the primary tumor with a surrounding margin of normal tissue and lymph node sampling of the draining nodal basin, provided that major functional/cosmetic impairment is not necessary.