Tracheobronchopathia osteochondroplastica is an unusual disease of obscure causation characterized by cartilaginous or bony outgrowths into the lumen of the tracheobronchial tree. Our retrospective review of 15 patients, 8 of whom were women, revealed a mean age of 63.5 years. The most common symptoms were cough (66%), hemoptysis (60%), dyspnea on exertion (53%), and wheeze (30%). Thirteen percent of the patients were asymptomatic. Chest radiography was not helpful in the diagnosis. Tracheal tomography revealed typical beaded intraluminal calcification in 4 of the 12 patients tested. Mirror laryngoscopy initially revealed the abnormalities in 30% of the patients, and bronchoscopy confirmed the diagnosis and determined the extent of the disease in all patients. Even though upper airway involvement has been thought to be uncommon, 40% of our patients demonstrated abnormalities of the larynx and upper trachea. Histologic confirmation of heterotopic bone formation was obtained in 60% of the patients. Pulmonary function tests showed mild obstructive lung disease. There were no deaths directly attributable to the disease. Treatments attempted included cryotherapy, laser excision, external beam irradiation, and bronchoscopic removal of the obstructing lesions.