Uterine carcinosarcoma (UCS: uterine malignant mixed Mullerian tumor, uterine MMMT, uterine MMT) is an uncommon but aggressive malignancy. Given that UCS is metaplastic carcinoma, the stage system is according to the 2009 FIGO (Federation of International Gynecology and Obstetrics) staging system for endometrial cancer. In addition, UCS is included into the classification of mixed epithelial and mesenchymal tumors of the uterus, based on the current 2014 World Health Organization (WHO) classification. The diagnosis of UCS is made in the presence of high-grade malignant epithelial and mesenchymal components typically showing a sharp demarcation. The principal treatment in early and locally advanced UCS is a complete and comprehensive staging surgery, including total hysterectomy, bilateral salpingo-oophorectomy, and systematic lymphadenectomy. For advanced-stage UCS, en-bloc debulking surgery is also beneficial for the patients. Multi-agent chemotherapy with and without radiotherapy is often prescribed for patients with UCS, because it might be associated with a better progression-free survival and overall survival. This review summarizes and analyzed the updated information of this highly aggressive disease.