Wegener's granulomatosis is an autoimmune condition resulting in the granulomatous vasculitis of small-to-medium-sized vessels, and is characterized by granulomatous lesions in the renal and respiratory systems. Cardiac involvement in Wegener's granulomatosis has been previously reported. However, involvement of cardiac valves is extremely rare. We present a patient with Wegener's granulomatosis with an extensive mitral mass extending to the aortic valve. (Ann Thorac Surg 2010; 90: 996-7) (C) 2010 by The Society of Thoracic Surgeons