Pediatric rhabdomyosarcoma is not exclusive to the head and neck. However, the unique anatomy of the head and neck requires special consideration and treatment modifications. The low incidence of these tumors has prevented the development of rigorous treatment protocols. Treatment strategies must be individualized on the basis of histopathologic subtype, prognostic indicators, tumor location, tumor extent, available clinical trial data, and hospital resources. The primary treatment of these tumors typically involves a combination of surgery, radiation, and chemotherapy. Advancements in surgical and radiotherapy techniques have reduced patient morbidity, whereas new chemotherapeutic protocols have improved local disease control and overall survival. Because of the infrequency and complexity of these tumors, patients may benefit from referral to centers with a comprehensive multidisciplinary team that has experience treating these tumors in the pediatric population. If possible, patients should be enrolled and treated on the current Children's Oncology Group protocol.