This disease is rare and was reported by Mounier-Kuhn in 1932. It is a congenital disease. It is associated with recurrent lower respiratory tract infections due to mucosal cilia dysfunction and difficulty in expelling endocrine secretions from the airways. Clinical symptoms are mostly chronic cough and sputum, dyspnea, and hemoptysis, associated with recurrent lung infections. Imaging manifestations are tracheal diameter (SAG) greater than 27 mm in men and 23 mm in women, significant tracheal and lung segmental bronchial dilatation, tracheobronchial wall softening, deformation and diverticulae. Bronchoscopy and biopsy remain the gold standard for this condition. Treatment of this syndrome is usually pharmacological with infection control and bronchoscopic with airway stenting and tracheobronchoplasty.