Ovarian carcinosarcoma is a rare and aggressive tumor with a poor prognosis. We report a case of ovarian carcinosarcoma and also review the literature. In 2000, a 63-year-old woman underwent optimal cytoreductive surgery for ovarian carcinosarcoma( International Federation of Gynaecology and Obstetrics[FIGO]stage III c[pT3cN0M0]). She received adjuvant chemotherapy with paclitaxel and carboplatin(TC). In 2005, a recurrent tumor was noted anterior to the sacrum. The patient had a complete response after 6 cycles of TC chemotherapy; however, a year later, the tumor recurred and was resected. In 2013, the tumor recurred adjacent to the right kidney and was surgically removed after a partial response to 3 cycles of TC chemotherapy. The pathologic findings included epithelial and non-epithelial components with histologic variation and differentiation; specifically, a leiomyosarcoma, cartilaginous tissues with cellular atypia, and a rhabdomyosarcoma were identified in specimens obtained during the first, second, and third surgical procedures, respectively. In keeping with the combination theory of histogenesis, the ovarian carcinosarcoma described herein may have originated from a monoclonal stem cell. The long survival of this patient is attributed to optimal cytoreduction during the primary operation, solitary recurrent tumors that were completely resected, and sensitivity to chemotherapy.