Background: Extraosseous Ewing's sarcoma is a rare condition. Its location in the uterovesical fold has not been reported in the literature. Case: A 32-year-old multiparous woman was diagnosed clinically and by ultrasound with a uterine fibroid 24 weeks in size. At laparotomy, there was a highly friable and vascular 30×16 cm mass present on the superior surface of the bladder and uterus, distending the uterovesical fold. The mass was enucleated piecemeal; rather, it came out piecemeal spontaneously after opening the uterovesical fold. The histopathological report indicated an extraskeletal primitive neuroectodermal tumor; tumor cells were positive for PAS and IHC, Vimentin, and CD99. She was advised to undergo chemotherapy and radiotherapy but was lost to follow-up after three cycles of chemotherapy. She returned after 6 months with a recurrence of the tumor and poor general health. Conclusions: Extraosseous Ewing's sarcoma is a very aggressive tumor, and recurrence is common after local excision. When it occurs in the uterovesical fold, it can mimic a uterine fibroid. (J GYNECOL SURG 28:425)
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