Objective: Wegener’s granulomatosis is a systemic vasculitis characterized by necrotizing granulomatous lesions mostly involving the upper and lower respiratory tract. The disease rarely causes parotid gland involvement and pneumothorax. We report a case of Wegener’s granulomatosis involving parotid gland, and complicated with a pneumothorax. Clinical Presentation: A 45-year-old man admitted with a 3-week history of painful left parotid gland enlargement and hemoptysis. On physical examination a painful and hard mass was detected on the left pre-auricular area. Cervical CT revealed a 2 × 1.5 cm hypodense lesion mimicking an abscess on the left parotid gland. Chest radiograph and thorax CT demonstrated nodular and cavitating opacities on the right and left upper zones. There were numerous erythrocytes in urine sediment. The drained pus material from the parotid abscess demonstrated only gram-positive cocci (Staphylococcus aureus). Two weeks treatment with teicoplanin resulted in no improvement. Meanwhile, parotid gland biopsy revealed necrotizing granulomatous inflammation. There was a sixfold increase in serum cANCA levels. With the diagnosis of Wegener’s granulomatosis, cyclophosphamide and prednisolone were initiated. However, 1 month later, pneumothorax developed as a complication of rupture of a cavitary lesion. Conclusion: Parotid gland swelling may be the initial presenting symptom of Wegener’s granulomatosis. It can be confused with infectious or malignant diseases of the gland, and the lung involvement may be complicated with pneumothorax.