A case of a 12-year-old girl with Ewing sarcoma of the third metacarpal bone is described. Following an incisional biopsy, radiation therapy and adjuvant chemotherapy were given. The girl is now 34 months postdiagnosis and 25 months off therapy in sustained complete remission and functionally normal right hand. Of the seven previously published cases only two are long-term survivors (one had disarticulation, and the other resection of the fourth and fifth rays).