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Wegener's granulomatosis, a clinicopathologic entity of unknown cause, is characterized by systemic necrotizing vasculitis. The disease primarily affects the medium and small muscular arteries in the upper and lower respiratory tract as well as the glomerular arterioles in the kidneys, but patients can present with a wide variety of signs and symptoms. In this article, the authors report a case of Wegener's granulomatosis diagnosed by open lung biopsy.