We report our experience in 8 patients with osteochondroplastic tracheopathy and consider the importance of its diagnosis y biopsy in order to confirm histology and if possible etiology, because specific treatment could change the course of the disease. Methods such as radiology, CT-scan, respiratory function tests only give diagnostic suspicion, which is a previous step before confirmation by bronchoscopy with biopsy. To date, with the exception of a few cases, the diagnosis is made by necropsy. Thus, this justifies our emphasis in the diagnosis of this rare disease when it is suspected in living patients.