In its classical form, Wegener's granulomatosis (WG) is characterized by acute granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis, and when untreated, mortality is high. The use of cyclophosphamide and prednisolon has improved the prognosis dramatically, while azathioprine and trimetoprim-sulfamethoxazole are of limited therapeutic value in these patients. Only 1/3 of subacute and chronic cases show renal impairment, and the prognosis is better in limited Wegener's granulomatosis without renal disease than in the classical form. This case-report and review takes up various new aspects of diagnosis, treatment and prognosis.