Ewing's sarcoma is a kind of bone and soft tissue tumor which is highly invasive and mainly occurres in chil-dren and adolescents. In recent years, combined chemotherapy, surgery and radiation therapy in treatment of Ewing's sarcoma, pa-tients’ prognosis and life quality have been significantly improved. However, over the past 20 years, the treatment of Ewing's sar-coma entered the platform period. The 5-year overall survival rate remained at 55%-75%. Multiple metastasis and recurrence are the main factors of poor prognosis and death. Chemotherapy, radiotherapy and molecular targeted therapy are still the main meth-ods for the treatment of Ewing sarcoma. The side effects, drug resistance and the use of the combination regimen of antitumor drugs have been plaguing the clinical workers. In order to improve the efficacy of chemotherapy drugs and reduce the toxic side ef-fects, Multi-disciplinary and multi-center clinical studies on Ewing's sarcoma patients who suffered from local control or recur-rence have been launched by Domestic and European and American countries. As an important supplementary mean for the treat-ment of Ewing's sarcoma, patients often appear a series of complications after radiotherapy, including the risk of local damage or secondary tumors. Therefore, it is necessary to further clarify the indications of radiotherapy and the timing of preoperative and postoperative radiotherapy. The specific chromosome translocation and the expression of the fusion gene EWS/FLI1 have been found in Ewing sarcoma. Nevertheless, the mechanisms that drive tumor relapse and metastasis remain unknown. Molecular target-ed therapy can be used to inhibit tumorigenesis and progression by regulating the upstream or downstream target genes of EWS/FLI1. In conclusion understanding of the current treatment status of Ewing's sarcoma, results of multi-center clinical trials and the-ory of genomics research will contribute to the design of new biological therapies so as to establish individualized treatment modali-ties. In this paper, we present a review on the progress of Ewing sarcoma chemotherapy, radiotherapy, molecular targeted therapy and immunotherapy.