Vascular leiomyosarcoma (LMS) are unique. The inferior vena cava (IVC) is the most affected organ (about 38% cases). We report the observation of a 50-year old woman who consulted for right upper quadrant pain. Imaging studies revealed a retroperitoneal mass that mimic a LMS of the IVC. The patient was operated. A resection of the IVC along with the tumor was performed without reconstruction. The management of LMS is surgical and depends upon the location and tumor characteristics.