A case of extraskeletal Ewing's sarcoma arising in the chest wall of a 15-year-old male is reported in this paper. His complaint was chest and back pain. Physical examination and laboratory data disclosed no abnormality. Radiographs and computed tomography revealed chest wall tumor, and resection of the tumor with a portion of the eighth rib was performed. Histologically, there was no involvement of the rib at all. The tumor tissue was composed of solidly packed tumor cells which were uniform in size and shape. Individual tumor cells were small, round-shaped, and had scanty, ill-defined cytoplasm. The diastase-digested, PAS positive material was found in the cytoplasm. NSE (neuron specific enolase) was positive, but EMA (epithelial membrane antigen) LCA (leucocyte common antigen), and keratin were negative. With these results, we got a diagnosis of extraskeletal Ewing's sarcoma. Combination chemotherapy with vincristine, cyclophosphamide, adriamycin, actinomycin D was administered after operation, and there is no evidence of recurrence after 12 months of follow-up.

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