Objective To explore the diagnosis and treatment of airway involvement in relapsing polychondritis. Methods The clinical data of two patients with relapsing polychondritis with airway involvement were reported and the relative literatures were reviewed. Results The two patients were both old males, with clinical manifestations of cough, dyspnea, and fever. They were misdiagnosed in a other hospital. The pulmonary function tests showed obstructive ventilatory impairemnt. On inspiratory CT, tracheal / tracheobronchial wall thickening and airway stenosis, with or without tracheal cartilage calcification were common findings. The tracheal cartilages thickeness and membranous wall were normal. On expiratory CT scans, functional abnormalities were identified such as tracheobronchomalacia. The patients were relieved by medication of corticosteroids or with immunodepressant. Conclusions The relapsing polychondritis with airway involvement is easy to be misdiagnosed. Chest CT examination is a valuable method for diagnosis of relapsing polychondritis. Corticosteroids and immunodepressant can improve the outcome.