Wegener granulomatosis (WG) is a necrotizing vasculitis that usually affects the respiratory tract, in association with kidney disease. Cardiac involvement is rare and silent in most cases, only becoming evident in necropsy studies. The authors report the case of a patient with WG, whose unstable angina was the first clinical manifestation, although the patient had suffered a previous unrecognized myocardial infarction. In most cases cardiovascular disease is clinically silent, but there may be symptoms indicating involvement of the coronary arteries, pericardium, myocardium, endocardium, cardiac valves, conduction system or thoracic great vessels. In most cases cardiac manifestations appear late in the natural history of GW. In the case reported, cardiac symptoms appeared as the first manifestation of the disease, a situation which we were unable to find described in the literature. The authors review the literature on cardiac manifestations of WG, together with recommendations for diagnosis and follow-up of this disease.