Tracheopathia osteoplastica is a rare disease characterized by submucosal cartilaginous or bony projections into tracheobroncheal lumen with sparing of the posterior membranous portion of tracheobroncheal tree. The cause of this disorder is unknown. In the past, a majority of the cases were discovered incidentally at autopsy. But recently, antemortem diagnosis is increasingly reported after the introduction of computed tomography and bronchoscopy. We report a case of extensive tracheopathia osteoplastica diagnosed antemortem by computed tomography, bronchoscopic examination and biopsy.