Background: Juvenile Granulosa Cell Tumor (JGCT) is a rare neoplasm in children. The clinical presentation usually includes a painful abdominal mass and pseudo-precocious puberty. The mainstay of treatment is surgical salpingo-oophorectomy for all cases, followed by platinum containing chemotherapy in advanced cases.
Case Report: We present a case of JGCT in a 2-year-old girl who presented with a history of precocious puberty for 1 year. The imaging studies showed an left ovary mass measuring 5.0. A laparotomy identifi ed peritoneal fluid and a tumor of the left ovary. Before the manipulation of the tumor, a sample of the peritoneal fluid was collected followed by a left salpingooophorectomy. The analysis of the peritoneal fluid identified neoplasic cells. The patient then received chemotherapy with cisplatinum, etoposide and ifosfamide.
Conclusions: This case emphasizes the importance of the peritoneal fluid sampling before salpingooophorectomy allowing correct surgical staging and treatment decision.

Keywords: Juvenile Granulosa Cell Tumor, Ascites, cytopathology, adjuvant chemotherapy