OBJECTIVE: Ovarian carcinosarcoma (OCS) is a rare malignancy accounting for only 1-4% of all ovarian cancers. The treatment of OCS is largely based on data from small case series and management of other histologic subtypes of epithelial ovarian cancer. We reviewed the literature pertinent to the pathology, pathogenesis, diagnosis, and management of women with OCS. METHODS: MEDLINE was searched in English for literature on OCS, focusing on the past 30years. Given the rarity of this tumor, studies were not limited by design or number of reported patients. RESULTS: Molecular, epidemiologic, genetic, and histologic data indicate that most OCS are monoclonal. Patients with OCS generally present with advanced stage disease. Most of the available retrospective studies support the role of cytoreductive surgery in the management of OCS, with optimal debulking associated with improved survival. Platinum-based chemotherapy is the current accepted adjuvant treatment. Given the limited data regarding the management of recurrent OCS, patients are usually treated similarly to women diagnosed with other subtypes of epithelial ovarian cancer. CONCLUSION: OCS represent a rare and aggressive histologic subtype of epithelial ovarian cancer. The goal of surgery is comprehensive staging in patients with early-stage disease and optimal cytoreduction patients with advanced-stage tumors. Platinum-based chemotherapy is the mainstay of adjuvant systemic treatment. Future studies are needed in order to elucidate the molecular basis for OCS and to evaluate the role of targeted therapy in its management.