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Renal Ewing sarcoma (RES) is an extremely rare disease. The standard treatment for this disease is lacking, and clinical experience needs to be accumulated. Here, we report a case of RES that rapidly developed to metastatic disease and was refractory to radiotherapy and chemotherapy; however, the case obtained a partial response based on Choi criteria by orally taking antiangiogenic drug apatinib. Our case suggests that apatinib may be a therapeutic option for RES.