Sex cord-stromal tumors of the ovary are the third most common types of neoplasms that develop in the ovary and account for about 5-8% of all ovarian malignancies. This group of ovarian neoplasms is derived from the sex cords and the ovarian stroma or mesenchyme. Granulosa-stromal cell tumors include granulosa cell tumor, thecoma and fibroma. The granulosa cell tumor is a low-grade malignancy and accounts for about 2-3% of all ovarian malignancies. Granulosa cell tumors have a prolonged natural history and tendency toward late relapse, reflecting their low grade biology. We present a case of juvenile granulosa cell tumor of the ovary with brief review of literature.