Uterine carcinosarcoma is a rare, metaplastic subtype of endometrial cancer comprised of two distinct malignant components – epithelial and mesenchymal, with phenotypic features. This tumor shows very aggressive behavior, including both local recurrence and distant metastases. Surgery, consisting of total hysterectomy, bilateral salpingo-oophorectomy and dissection of pelvic and para-aortic lymph node, with detailed examination of the entire abdominopelvic cavity and maximal cytoreduction of the lesions, is the principal treatment. The optimal postoperative therapy has not been determined, and is individualized. In a randomized trial a postoperative radiotherapy was shown to improve local control but no survival benefi t. High rate of distant metastases suggests a potential role of the systemic therapy. However, the benefi t of postoperative chemotherapy in high-risk patients has not been confi rmed in randomized studies. This method, in addition to palliative radiotherapy and surgery, is used in recurrent and advanced disease. Currently, chemotherapy including the combination of paclitaxel with ifosfamide or carboplatin is considered the most effective regimen, with the latter having a better toxicity profile.