Adenoid cystic carcinoma (ACC) is a rare malignant epithelial tumor that predominantly originates in the salivary glands. Primary ACC of the tracheobronchial tree is extremely rare. We report two new cases of central airways primary ACC: a 58 year-old male with an ACC of the left main bronchus who underwent a pneumonectomy with node dissection, and a 52 year-old female with proximal tracheal ACC presenting as asthma treated by surgical resection and a postoperative radiotherapy. Primary ACC of the tracheobronchial tree is often misdiagnosed given the non-specific clinical presentation. An early diagnosis is essential to ensure good outcomes. An interdisciplinary treatment is required based especially on surgery and radiotherapy.