Background: Although rhabdomyosarcoma is the most common soft-tissue sarcoma in children, primary pure ovarian rhabdomyosarcoma is extremely rare. Case: A 10-year-old girl presented with a gradually increasing abdominal mass for 2 months, with no other apparent soft-tissue swelling. Laparotomy revealed a large solid left ovarian tumor with no ascites and no lymphadenopathy. On histology and immunohistochemistry, a diagnosis of rhabdomyosarcoma was made. Postoperatively she received chemotherapy and was doing well after 16 months follow-up. Conclusions: Rhabdomyosarcoma is an aggressive neoplasm with a tendency for local infiltration and metastatic spread. Treatment requires surgery followed by adjuvant chemotherapy and/or radiotherapy. (J GYNECOL SURG 28:428)
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