Wegener granulomatosis is a rare systemic idiopathic disease characterized by involvement of small vessels - medium and small arteries, venules, arterioles and ocasionally large arteries. This disease has predilection for the upper and lower respiratory tract and the kidney, with granulomatous inflamation and necrosis. Clinical manifestations and organ involvement of the disease vary widely. Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal. The authors present a case of a 33 year-old female, with severe disease, but with good outcome, after adequate diagnosis and treatment.