Intravenous leiomyomatosis (IVL) is a rare smooth muscle tumor. It is characterized by nodular masses of histologically benign-looking smooth muscle cells growing within the uterine and extrauterine venous system. There are no clear guidelines regarding surgical approach and management of IVL in the literature. We would like to present an IVL case with extension to the right pelvic cavity and discuss the case in accordance with the current literature.