Wegener's granulomatosis (WG) is a systemic inflammatory disease whose histopathologic features often include necrosis, granuloma formation, and vasculitis of small-to-medium-sized vessels. We report a new case of Wegener's granulomatosis occurring in a 41-year-old man who was admitted for respiratory symptoms, fever, asthenia, night sweats and weight loss since one month. Chest X-ray and computed tomography showed multiple bilateral pulmonary excavated lesions. A trransparietal lung biopsy was performed. Histological examination showed an area of necrosis, surrounded by a palisade of histiocytes. Some of the histiocytes were multinucleated and formed true granulomas within the wall of the adjacent pulmonary artery. Serology was strongly positive for ANCA. A diagnosis of Wegener granulomatosis was conducted and the patient was started on cyclophosphamide and methylprednisolone as an initial treatment, with a favorable evolution.