Wegener's granulomatosis is a necrotizing vasculitis disorder that usually presents with clinical involvement of the upper and lower respiratory tract in association with renal disease. Although Wegener's granulomatosis can affect other systems, such as the eye, skin, joints, muscle, nerves, gastrointestinal tract and heart, cardiac involvement is traditionally believed to be rare, even though coronary vasculitis can be demonstrated at postmortem examination. We report a patient who has both respiratory and renal involvement who died unexpectedly following a silent myocardial infarct after a period of clinical improve induced by treatment with cyclophosphamide and prednisone.