Tracheobronchopathia osteochondroplastica is a rare airway disease of unknown aetiology. Due to overlapping symptomology and lack of awareness, the condition is often missed resulting in unnecessary medical or surgical treatment. A male patient presented with a long-standing history of hoarseness and had earlier received treatment for bronchial asthma and tuberculosis. On evaluation, he had typical submucosal calcified nodules distributed throughout the trachea sparing the posterior membranous part. Although the biopsy confirmed the diagnosis of tracheobronchopathia osteochondroplastica in our case, histopathological examination is not always needed to make this diagnosis. Our patient has been kept under conservative management and is having non-progression of disease at 1-year follow-up. After having reviewed the literature related to pathophysiology and management of tracheobronchopathia osteochondroplastica, we emphasise on the fact that the treating physicians' awareness about this condition is the key to its diagnosis and management.
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