Wegener's granulomatosis is a type of vasculitis characterized by necrotizing granulomatosis respiratory tract lesions and necrotising glomerulonephritis. Nasal, lung and renal biopsies and positive antineutrophil cytoplasmic antibody (c-ANCA) analysis is helpful in diagnosis. Early diagnosis and treatment is critical in prognosis. A 42 years-old male had dyspnea, coughing, hemoptysis, fatigue, loss of appetite, night sweating and arthralgia. Violaceous palpable, purpuric lesions were detected on the sublingual region of the mouth. On chest X-ray, there was nonhomogeneous infiltration in the parenchyma of both lungs. There was alveolar density involving upper lobes of both lungs detected in thoracal computerized tomography as well as patchy densities on the right lung upper-middle lobes. A nasal mucosa biopsy showed strongly destructive vasculitis. c-ANCA test was positive. We report an unusual case with Wegener's granulomatosis, characterized by a rare presentation of tongue involvement and atypical lung radiology with alveolar opacity.