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Extraskeletal Ewing sarcoma (EES) of the extremity is uncommon, and only a small number of reported cases have been devoted to the upper-extremity. A 65-year-old woman presented with a recurrent EES, a highly malignant tumor, involving the ulnar nerve at the right elbow region which was initially suspected as a benign soft tissue tumor, schwannoma, thus marginal excision had been performed. Due to its malignant behaviour, we treated the recurrent lesion with wide excision and reconstruction combined with chemotherapy. Histological evaluat