Uterine leiomyosarcoma (uLMS) is a relatively rare uterine cancer that behaves aggressively, with high lethality, even in the setting of early-stage disease at diagnosis. Given the propensity for metastases even in apparent early-stage disease, and relative resistance to chemotherapy and radiation, uLMS remains a challenge to treat. In recent years, targeted therapies have been assessed in an effort to provide additional management options to reduce recurrence and improve survival. However, while clinical trials have shown targeted therapies to have activity in uLMS, survival benefits have not been observed. Given the overall meager impact of systemic therapy and radiation on survival in uLMS, surgical management remains an important component of multi-modality treatment.