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Gata Haydarpaşa Eğitim Hası. İç Hastalıkları Kliniği Wegener's Granülomatosis is a fatal systemic disease of unknown etiology, characterized by necrotizing and granulomatous vasculitis of the upper and lower respiratory tracts together with necrotizing glomerulonephritis. Variable degrees of disseminated vasculitis involving small arteries and veins may lead to symptoms and signs in such a broad spectrum that may complicate the diagnosis. One of the distinct features of this formerly universally fatal disease is the excellent and long term remission achievements with Cyclophosphamide in over 90 percent of patients. In this article a case of Wegener's Granulomatosis manifested symptomatic pulmonary infiltrates, radiologically resembling metastatic carcinoma to the lung and mucocutaneous necrotizing vasculitis is presentented. Complete remission has been achieved with Cyclophosphamide and duration of remission is being followed. Keywords :