Spontaneous pneumothorax in Wegener granulomatosis (WG) is uncommon. Three cases of pneumothorax that occurred early in the course of this vasculitis are reported. In the first patient, the disorder was disclosed by a pyopneumothorax. In the second patient, a rupture of the subpleural cavitary nodule into the pleural space was observed. In the third patient, the pneumothorax was discovered at the same time as a pulmonary hemorrhage. The three patients improved with immunosuppressive therapy. In WG, the pulmonary nodules are predominantly in the subpleural location, which entails the risk of pneumothorax and therefore require particular attention.