Tracheobronchopathia osteochondroplastica (TPO) is an idiopathic disease of the trachea and major bronchi characterized by multiple submucosal osteocartilaginous nodules. The nodules may be either focal or diffuse, and typically spare the membranous wall of the airways. Symptoms are non-specific, and include dry cough, dyspnea, recurrent respiratory infections and occasionally hemoptysis. TPO is rarely considered as a diagnosis in part due to lack of awareness among clinicians. The diagnosis can be based on a typical bronchoscopic appearance and generally does not require biopsy of the lesions. When available, histology reveals bone formation within the submucosa with normal overlying respiratory epithelium. TPO is a benign disorder, marked by a generally favorable clinical evolution. There is currently no established treatment for the removal of airway nodules, or the prevention of further tissue growths. Interventional bronchoscopy techniques have a role in the relief of symptomatic airway obstruction, when indicated.