BACKGROUND Granulomatosis with polyangiitis (GPA)/Wegener's granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss' syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis. CASE REPORT We present the case with a 56-year-old male diagnosed with ANCA associated vasculitis, who began experiencing respiratory symptoms primarily thought to be respiratory tract affection. These symptoms worsened, and an echocardiography revealed heart failure with decreased left ventricular ejections fraction (EF=30-35%) and a severe insufficiency of the aortic valve. The patient underwent aortic valve replacement with symptomatic relief. Pathological examination of aortic valve resectates revealed inflammation and thickening of the aortic cusps. CONCLUSIONS Patients with ANCA associated vasculitis can rarely present with valvular inflammation causing severe regurgitation. The aortic valve can be involved, although cases have also described mitral valve involvement and both valves simultaneously. In patients with ANCA associated vasculitis a severe worsening of dyspnea can be caused by exacerbation of pulmonary involvement, but severe valvular disease should also be considered.