Granulosa cell tumors of the ovary are sex cord stromal tumors. Sex cord stromal tumors make up 8% of ovarian tumors. Ninety percent of sex cord stromal tumors of the ovary are granulosa cell tumors. There are two types of granulosa cell tumors: juvenile and an adult type (Jamieson and Fuller. Molecular pathogenesis of granulosa cell tumors of the ovary. Endocr Rev 2012;33:109–44). This article provides a literature review outlining histopathology, immunocytochemistry and cytogenic characteristics of adult granulosa cell tumor of the ovary and a case report of synchronous adult granulosa cell tumor of the ovary and endometrial adenocarcinoma.