Leiomyosarcoma of the inferior vena cava is a particularly rare tumour, originating from the smooth muscle of the vessel wall. The authors describe the case of a female patient admitted with a picture of anorexia and weight loss, accompanied by epi- and mesogastric pain. Preoperative examinations revealed the presence of a mass of considerable size originating from the inferior vena cava. The patient was submitted to surgery consisting in removal of the mass and of part of the wall of the vena cava. A review of the literature confirms the rarity of this tumour and demonstrates that optimal anatomical knowledge is absolutely indispensable for the management of this pathology.