ABSTRACT
INTRODUCTION: Extraskeletal Ewing's sarcoma is a rare malignant tumor of mesenchymal origin, which is histologically similar to primary osseous Ewing's sarcoma. It has been well described in deep soft tissues. However, location in cutaneous or subcutaneous tissue has rarely been reported. Being seen principally in children, it can be seen, rarely, in old men. CASE PRESENTATION: We present a case of large primary cutaneous Ewing sarcoma within the left thigh of a 35-year-old woman, without osseous involvement. Physical examination. Histologically, it was a small round cell tumor that marked strongly for CD99. The diagnosis of cutaneous Ewing sarcoma was performed. DISCUSSION: The things that distinguish our case are that it is the first case in Syria, in addition to the size of the large tumor with diameters of 15 × 20 cm, and it is in a 35-year-old woman. CONCLUSION: Ewing sarcoma is a rare malignant small round cell tumor of the skin and subcutaneous tissue. It should be differentiated from other cutaneous neoplasms composed of small round cells.