Between 1980 and 2007, five patients were pathologically diagnosed as tracheobronchial adenoid cystic carcinoma (ACC). All five patients were women aged 37-67 years. Four tumors were located in the larger airways, and one tumor was located in the peripheral lung. The following operations were done: bronchoplastic procedures in three (carinal resection with doublebarreled carinoplasty in one, sleeve right pneumonectomy in one, sleeve middle lobectomy in one), left pneumonectomy in one, and left upper lobectomy in one. Three of the five patients have survived for 172, 144, and 10 months after surgery, respectively. The best local treatment for ACC of the major airway is considered to be sleeve resection of the trachea or bronchus in an area where airway reconstruction may not be disturbed and to add postoperative irradiation when there is residual carcinoma at the stump. However, it seems controversial to recommend adjuvant radiotherapy in all patients undergoing resection.