A 54-year-old woman was admitted to our hospital complaining of postcoital bleeding. Sonography of the abdomen showed a 8.2 x 8.9 cm-sized solid heterogeneous mass occupying the cul-de-sac, which appeared to be in no way connected with the ovary. On exploratory laparotomy, the tumor mass protruded from the posterolateral retroperitoneum of the pelvic cavity and severely replaced the uterus and adnexa with the outer surface being grossly intact. It grossly measured 10 cm in maximal diameter. The histologic features closely resembled those of ovarian granulosa cell tumor. The primary extraovarian granulosa cell tumor is extremely rare such that in the English literature only 7 cases have been reported to date. Of those granulosa cell tumors are especially rare and only two cases have been reported to arise from retroperitoneum. We herein present a case of retroperitoneal granulosa cell tumor with special regard to differential diagnosis from other solid tumors with similar histology.