Tracheobronchopathia osteochondroplastica (TO) is a rare disorder of the large airways characterized by the development of submucosal cartilaginous and bony nodules. The nodules involve the anterior and lateral walls and typically spare the posterior membranous wall. The clinical presentation of TO is variable and ranges from incidental diagnosis in asymptomatic patients during workup or management for unrelated medical problems, to devastating disease with central airway obstruction. Bronchoscopy remains the gold standard for diagnosing this condition. Radiographic studies play an important role in suggesting the diagnosis of TO and in the follow-up of this condition. The treatment of TO is usually symptomatic. with emphasis on the management and prevention of recurrent respiratory infections. Bronchoscopic or surgical treatment is usually reserved for symptomatic patients with severe airway narrowing and airflow obstruction.