Wegener’s granulomatosis (WG) is a necrotizing vasculitis that mainly affects the respiratory tract and kidneys. Of note, involvement of the heart is being increasingly recognized in these patients. Cardiac manifestations can arise from the coronary arteries, pericardium, myocardium, endocardium, valves, conduction system and great vessels, but in most cases cardiac involvement is clinically silent. Consequently, a regular cardiovascular evaluation, including echocardiography, should be performed in every patient with WG, while patients with WG flare should be closely monitored for cardiac complications even in the absence of specific symptoms.