Wegener's granulomatosis is a necrotizing granulomatous vasculitis often characterized by involvement of the upper respiratory tract, lungs and kidney, although any organic system can be affected. We present the case of a female patient with Wegener's granulomatosis diagnosed by biopsy of the kidney, and severe gastrointestinal bleeding with fatal course. Endoscopic findings of the colon and the histopathology of the biopsy are discussed.