Granulomatosis with polyangiitis (Wegener's granulomatosis) is a rare and a serious systemic vasculitis with preferential involvement of the upper and lower respiratory tract, eyes skin and kidneys. The diagnosis is established based on the clinical and paraclinical exams. Granulomatosis with polyangiitis may affect any airway segment, determining inflammation, ulceration, pseudomembranes, tracheobronchomalacia, cartilage destruction and laryngeal-tracheo-bronchial stenoses.