Background: Tracheobronchopathia osteochondroplastica (TBPOCP) is an uncommon benign condition affecting the lumen of tracheobronchial tree and characterized by abnormal chondrification and ossification. TBPOCP is more frequent than it has been reported, as it can be asymptomatic or present with non-specific respiratory symptoms. Aims: In this article, we provide a review of the English literature on the condition and discuss its clinical features, general principles, diagnostic approaches and current treatment recommendations for TBPOCP.