The occurrence of true extrarenal Wilms' tumor is extremely rare. The most frequently noted extrarenal sites are the retroperitoneal and inguinal regions. In the female genital tract, the occurrence of Wilms' tumor has been documented in the uterus, endocervix, and ovary in isolated case reports. In this article the authors describe a case of ovarian Wilms' tumor in a 3.5-year-old girl. Her abdominal ultrasound scan and computed tomography scan showed a solid mass with cystic components on the left lower quadrant. Total excision was performed with left salpingo-oophorectomy. There was no other mass and also no evidence of metastasis. To the best of the authors' knowledge, this patient is the first reported case of primary ovarian Wilms' tumor arising in childhood. J Pediatr Surg 37:127-129. Copyright (C) 2002 by WB. Saunders Company.