Adenoid cystic carcinoma (ACC) is a tumor that occurs most commonly in major and minor salivary glands and progress slowly. It has also been described in the breast, skin, uterine cervix, upper aerodigestive tract and lung. Trachea and main bronchus are the localizations where it is most commonly seen in the respiratory system. In this study, 10 cases that were clinically diagnosed and followed as ACC between 1988 and 2000 were evaluated retrospectively. There were 5 male and 5 female patients and the mean age was 46 years (22-62 years). Tumors were localized in trachea (5 cases), in main bronchus (2 cases), and in lober bronchus (3 cases). Operative approachments were resection of trachea (2), tracheal resection + carinoplasty (2), right sleeve upper lobectomy (2), left pneumonectomy (2), biopsy + tracheostomy (1) and middle lobectomy (1). In five of the patients microscopically positive resection margin was established. ACC is one of the rare tumors of the pulmonary system. Some patients with ACC could be treated for prolonged periods for an erroneous diagnosis of asthma because of the tracheal or main bronchial obstruction. Therefore, these patients may be admitted to the emergency department of chest disease and thoracic surgery. Surgery is the primary management of ACC. For the patients with unresectable tumors, primary radiotherapy (RT) or RT after Nd-YAG laser ablation may provide satisfactory results.