Objective To investigate the clinical efficacy and prognosis of juvenile rhabdomyosarcoma. Methods The clinical data of 37 patients with rhabdomyosarcoma admitted to our hospital from January 2010 to December 2017 were analyzed. Results There were 22 males and 15 females, ranging in age from 13 to 30 years, with a median age of 22 years. According to the staging criteria of the American rhabdomyosarcoma research group (IRS), there were 7 cases in stage I, 4 cases in stage II, 6 cases in stage III, and 20 cases in stage IV. All patients were confirmed by histopathological biopsy and immunohistochemistry. There were 20 cases of embryonal type, 13 cases of acinar type and 4 cases of pleomorphic type. There were 2 cases of simple operation, 2 cases of radiotherapy alone, 13 cases of systemic chemotherapy only, and 20 cases of surgery combined with radiotherapy and chemotherapy. Follow up to March 30, 2018, 9 cases survived and 28 died. Conclusion The common pathological type of juvenile rhabdomyosarcoma is embryonal type. It is mainly treated with surgery, radiotherapy and chemotherapy, but its malignant degree is high and the mortality is high. It is expected to improve the survival rate by multidisciplinary comprehensive cooperation. The prognosis is likely to be related to the tumor staging and pathological types.