Intravenous leiomyomatosis of the uterus is a rare tumor that presents with benign histological features. It is characterized by intravenous proliferation of smooth muscle cells originating from the uterus that sometimes extends to the inferior vena cava and the right heart. It may spread elsewhere, usually to the lung. The first case of intravenous leiomyomatosis was described in 1896 by Birsh-Hirshfeld, and only a few cases have been reported since then. Its pathogenesis and optimal treatment have not yet been established. We report a case of metastasizing leiomyomatosis found to have multiple nodular densities in both lower lung fields seven months after myomectomy. In another case the leiomyomatosis was confined to the pelvis after a laparoscopy assisted vaginal hysterectomy, the patient is alive without evidence of disease. Here we provide a detailed report of two cases of intravenous leiomyomatosis of the uterus with a brief review of the literature.