We report a 58-year-old man who underwent surgical treatment of primary lung cancer arising from the wall of a giant bulla. Chest roentgenography and computed tomography revealed multiple emphysematous bullae in the bilateral upper lobes, and a right upper giant bulla with a mass measuring 6 cm arising on the bulla wall. Right upper lobectomy was performed, the postoperative pathological diagnosis was large cell carcinoma arising from the wall of a giant bulla. Although the postoperative course was uneventful and he was discharged, he underwent partial resection of the jejunum for recurrence of carcinoma in the jejunum, and postoperative chemotherapy, and he was alive 20 months after that operation. In general, patients with both pulmonary bullous disease and primary lung cancer have a very poor prognosis, because they receive treatment when the tumor is at an advanced stage. On the basis of our review of the literature, we recommend that middle-age male patients with a giant bulla who smoke should have annual chest roentgenography and/or chest computed tomography to screen for lung cancer arising in or close to the bullous disease, and that a giant bulla should be resected in patients older than 50 years because of the high incidence of coexisting cancer and bulla, to improve the prognosis of this disease.