Granulomatosis with polyangiitis (Wegener) is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. We present a case with pulmonary and kidney involvement, which progressed to severe disease. We present the case of a 16-year-old boy, previously healthy, who was admitted to the hospital with one month of nasal discharge, cough and vomiting and 4 days of fever. Prior to this admission he presented respiratory