A 3-year old girl with juvenile Granulosa Cell Tumor (JGCT) was reported and 11 cases with JGCT taken from Japanese literatures are reviewed. The patient, presenting with abdominal pain and distension, was referred to our hospital. An ultrasonic examination and CTscan demonstrated tumors at the bilateral ovaries and right adrenal region. Bilateral salpingo-oophorectomy and partial resection of the right adrenal tumors was carried out. Subsequently, she re-cieved several courses of chemotherapy consisting of cyclophosphamide, vincristine, adreamycin and cisplatin. However, no obvious therapeutic response was obtained and the patient died 4 months after her initial presentation. Although, the prognosis of JGCT is reported to be better than that of adult type. But it may be unfavorable in advanced cases with JGCT. Clearly, more effective treatment for advanced cases must be established.