The paper describes an autopsy case of Wegener's granulomatosis with a rare variant of a cardiac lesion--the concurrence of aortic valvular disease (aortic regurgitation), fibroplastic endocarditis, and complete transverse blockade, followed by pacemaker implantation. The direct cause of death in the female patient was multiple organ dysfunction: progressive chronic heart failure, adult respiratory distress syndrome, acute hepatorenal failure, and pancreonecrosis. The data available in the Russian and foreign literature on investigations dealing with cardiac pathology in patients with Wegener's granulomatosis are analyzed.