We present the rare case of a 61-year-old female patient who was submitted in the hospital with metrorrhagia and pelvi-abdominal pain. Echographic examination revealed an heterogeneous uterine mass measuring 190/130/110 mm. Therefore, total hysterectomy with bilateral ooforectomy was performed. Grossly, the uterus presented a tumoral mass with areas of hemorrhage, necrosis and abundant mucoid degeneration. On light microscopic examination a malignant neoplastic proliferation with features of myxoid leiomyosarcoma was noted. In this paper, we presented this rare form of leiomyosarcoma with an emphasis on its particularities that have not been mentioned in the literature.