Uterine leiomyosarcomas are rare and have a poor prognosis, particularly at the metastatic stage. The rarity of this tumor justifie that patients be referred to referral centers afin order to have optimal diagnostic and therapeutic management. Anatomopathologic diagnosis is based on the demonstration of three features: necrosis, cytologic atypia, and mitoses within a smooth muscle proliferation. Chemotherapy probably has a place in the adjuvant management. In locally advanced or metastatic forms, the prognosis is poor, with an overall survival of about 12-14 months despite first-line polychymic therapy that gives response rates of about 30%. Anti-angiogenic drugs and hormone therapy have their place in the therapeutic arsenal. Multimodal management can improve the prognosis of some patients