We describe a case of unsuspected infrahepatic interruption of the inferior vena cava with hemiazygos continuation in a 67-year-old man presenting with chest pain and evidence of mitral regurgitation. He had no persistent superior vena cava, with the hemiazygos draining directly into the right superior vena cava. Polysplenia and severe mitral prolapse were also present: the latter may represent more than an incidental finding in this condition. This malformation may deserve consideration in adults undergoing femoral right heart catheterization. Chest radiographic studies are the basic clue to the diagnosis.