Retroperitoneal leiomyosarcomas are uncommon tumors, with approximately 300 documented cases in the literature. Management necessitates complete surgical resection in order to offer patients a chance at long-term cure. Resection often presents a challenge as these tumors are often large, involving adjacent structures, and may require reconstruction of the inferior vena cava (IVC). In this article, we will present background information on retroperitoneal leiomyosarcomas and the technical aspects of surgical resection and vascular reconstructive options of the IVC.