Uterine sarcomas comprise a group of rare tumors with different tumor biology, natural history and response to treatment, contain just 3-7% of total uterinemalignancies and about 1% of all gynecologic cancers. Although they cause importantpart of women death due gynecologic cancers. These tumors have aggressive behaviorand high recurrence rate, even when confined to the uterine corpus at the time ofdiagnosis. The most common of uterine sarcomas is leiomyosarcoma. The incidence ofleiomyosarcoma is increased after age 50. Traditionally, carcinosarcomas were namedas Malignant Mixed Mullerian tumor (MMMT), but in recent classification accordingto their pathologic structure and its behavior, these tumors are classified as carcinomas. The rare group of sarcomas is endometrial stromal sarcoma (ESS), which occurres inyounger women. In a medical studies search from 2000 to 2017, all kinds of uterinesarcomas, pathologic diagnostic methods, primary treatment and supportive treatmenthave been analyzed. Last histological classification is based on FIGO 2009 and WHO. According to such classification, sarcomas divided into three subtypes: leiomyosarcoma, endometrial stromal sarcoma and carcinosarcomas. Diagnosis of sarcoma beforetreatment and discrimination from benign myoma by current diagnostic methods isdifficult. Preoperative endometrial sampling identifies only 25% of sarcomas. It may bethe myometrial origin of tumor. Currently, MRI, ultrasound and PET scan may be usedfor the diagnosis of tumor. The gold standard of treatment is complete and intactresection of tumor considereing free margins. In advanced or recurrence disease, cytoreductive surgery followed by chemotherapy is the choice of treatment. Iftechnically it is not possible or there are extra abdominal metastases, palliativechemotherapy should be considered. Combination of gemcitabine and docetaxel is anacceptable choice. Recent studies are going to approve the effective role for targetedagents with or without cytotoxic chemotherapy in these group of aggressive tumors. The only drug in this group has approval is pazopanib. However, it did not achievedacceptable responses in phase I, II studies. As regards of tumor biology andinappropriate response to chemotherapy and radiotherapy, sarcoma have poor prognosisin all stages.