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A case of Wegener's granulomatosis is described, with special attention focused on the typical histologic findings and involvement of both middle ear and lung. The patient is a 37-year-old man presented with four-month history of cough and sputum. He had a past history of surgery of both ears because of otitis media followed by left facial palsy. Chest radiographs showed variable sized ill defined nodules in both lower lobes with internal airspace consolidation. Histologic preparations of the open lung biopsy specimens demonstrated a diffusely scattered palisading micro and macrogranulomas with central focus of neutrophils and necrotic collagen surrounded by histiocytes, histiocytic giant cells. Fibrinoid necrosis involved blood vessels and lung parenchyma. Chronic inflammation, diffuse granulation tissue formation and irregular fibrosis are also found in the lung parenchyma. The histologic findings of middle ear which was previously biopsied showed scattered palisading ill defined microgranulomas mixed with fibrotic tissue.