Wegener's granulomatosis (granulomatosis with polyangiitis) is a form of vasculitis of small-to-medium-sized vessels and associated with diffuse anti-neutrophil cytoplasmic antibodies (cANCA). Cardiac involvement is not uncommon with 6–25% of unselected patients and up to 44% of patients with severe renal involvement. We report a 23-year-old man with Wegener's cardiomyopathy with 25% ejection fraction. The overall mortality rate of Wegener's granulomatosis with cardiac involvement has been reported to be between 15 and 45%. So it is important to keep in mind that cardiac examination is a must to detect if cardiac involvement is present.