Tracheobronchopathia osteochondroplastica (TO) is a rare benign disorder affecting the trachea and occasionally the bronchi. We report a case of TO presenting as a respiratory insufficiency. Chest radiograph revealed an irregular narrowing of the intra thoracic trachea and a parenchymal consolidation of the left lower lobe. Magnetic resonance examination of the chest showed a diffuse irregular thickening of the trachea and central bronchi, which had an intermediate signal intensity with punctiform low signal intensity suggesting calcifications and no contrast enhancement. The diagnosis was confirmed by bronchoscopy and biopsies.