The primary ANCA associated vasculitides, Wegener's granulomatosis (WG), Churg Strauss syndrome (CSS) and microscopic polyangiitis (MPA), frequently affect the ENT region. For several reasons WG is of special significance for the otorhinolaryngologist. First, disease activity limited to the upper respiratory tract (localized WG) often proceeds the systemic vasculitis (generalized WG). The early diagnosis therefore has decisive consequences for stage adapted therapy. Second, in most cases (nearly 80%) WG is diagnosed histologically on biopsy specimens from the ENT region. During the initial phase of WG this is of diagnostic relevance, because at this stage the serologic parameters (acute-phase proteins) usually have a normal value and PR3-ANCA is (still) negative in 2/3 of the patients. Third, in many cases recurrences reveal increased activity in the ENT region, or start in this area. Clinically in most cases chronic rhinosinusitis with crusting and epistaxis is seen, sometimes with septal perforation and/or saddle nose. Apart from this there are often unclear middle ear symptoms with recurrent effusions and the inner ear is sometimes also affected. Laryngeal manifestations are typically located in the subglottic area and lead to subglottic stenosis. In the differential diagnosis, diseases in which epitheloid cell granulomas occur, such as sarcoidosis and TBC, need to be considered, but also foreign body granulomas and fungus diseases. Finally malignant tumours, especially malignant lymphomas, have to be ruled out.