Leiomyosarcoma of the inferior vena cava is an extremely rare malignancy originating from the smooth muscle of the vessel wall, with only a few hundred cases reported in the literature. There are no clear guidelines for treatment, but surgical resection is currently the only curative option. Further research is needed to better understand the disease and guide its management. We report a case of a 39-year woman who presented to the emergency room with a four-day history of nonspecific abdominal pain, nausea and fever. An abdominal computed tomography revealed a mass in relation with the inferior vena cava and hepatic nodules. Histological examination proved it to be a leiomyosarcoma of inferior vena cava with liver metastases. Key Words: Leiomyosarcoma, Inferior vena cava, Sarcoma.