Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of children. It is a high-grade malignancy and aggressive. Factors, such as histological type, stage, age and molecular marker are important for stratification and treatment, even associated with the prognosis. Currently, with multidisciplinary treatment, patients with RMS have achieved a long-term survival rate of over 70%; the long-term survival rate of low-risk RMS is up to 90%; future studies should focus on decreasing the treatment intensity and long-term toxicity; standard chemotherapy regimen for intermediate-risk RMS is still VAC (Cyclophosphamide + Vincristine + Actinomycin ). The improvements of surgery and radiation therapy technology will help improve the local control rate and preservation of organ function in patients.