Uterine sarcoma is a rare malignancy, accounting for 3–8% of all malignant tumors of the uterus. As a rule, patients are treated in reference centers by multispecialty diagnostic-therapeutic teams. Sarcoma of the uterus originate basically from two tissues – uterine muscle and endometrial stroma and constitute a heterogenous group of tumors. A standard of local treatment of all these tumors is transabdominal hysterectomy. High recurrence rate supports the rationale for adjuvant treatment, although to date no effect of adjuvant radiotherapy on overall survival and local recurrence rate has been proven. Some reports concerning stromal uterine sarcomas suggest an improved survival in patients undergoing postoperative hormonal therapy. The role of chemotherapy in adjuvant treatment of leiomyosarcomas remains to be defined. In the treatment of advanced stromal uterine sarcomas, there is a place for hormonal therapy (progesterone analogs, aromatase inhibitors and GnRH analogs). Treatment of far-advanced/ metastatic leiomyosarcoma cases and non-differentiated sarcomas is similar to systemic treatment of other soft-tissue sarcomas. The topic of this paper are current recommendations concerning diagnostic and therapeutic management of uterine sarcoma patients. Based on own experience confronted with literature data, we present current classifications, epidemiology, risk factors and generally accepted therapeutic protocols used to treat these malignancies.