Objective: Wegener’s granulomatosis (WG) is a necrotizing vasculitis that mainly affects the respiratory tract and kidneys, but can also affect other systems such as the eye, joints, skin, muscles, nerves, and gastrointestinal tract. Cardiac involvement is traditionally believed to be rare. We report a patient with silent myocardial infarction (MI) and review previously reported cases showing this association. Methods: A Medline database search of cases published between January 1978 and July 2008 both in English and Spanish, reporting silent MI complicating WG, was conducted. Results: We describe a typical patient with WG who had both respiratory and renal involvement and died unexpectedly following a silent MI after a period of clinical improvement induced by treatment with prednisone and cyclophosphamide. We report necropsy findings and the association with 5 additional cases of WG with silent MI reported in the literature. Conclusions: Clinicians should be aware of potential cardiac involvement due to WG. Careful evaluation of each patient, with or without cardiac symptoms, using ECG, echocardiogram, and myocardial enzymes is prudent.