Uterine leiomyosarcoma is a rare form of sarcoma with limited treatment options and a high potential for distant recurrence. At the time of diagnosis, swift action should be taken to initiate treatment. Options for treatment include surgical debulking and disease staging, radiation therapy, and/or chemotherapy. The purpose of this article is to review the disease epidemiology, presentation at diagnosis, surgical staging, and prognosis. Treatment options and the role of targeted therapies are discussed in addition to the various nursing implications associated with management of the disease.