Extraskeletal Ewing's sarcoma is a very rare tumor which was first reported by Angervall and Enzinger in 1975. The common sites of extraskeletal Ewing s sarcoma are bony structures of lower extremities, paravertebral region, and pelvis, but rarely chest walL Microscopically, extraskeletal Ewing's sarcoma is indistinguishable from the Ewing's sarcoma of bone. We present here a case of extraskeletal Ewing's sarcoma of the left lateral chest wall in a 19-year-old male. Wide extirpation and postoperative combined chemotherapy were done, and we discuss the clinical picture, histopathology, therapeutic management, and prognosis with review of the literature.