Tracheobronchopathia osteochondrodysplasias is a benign and rare chronic disease, whose etiology remains obscure. It is characterized by the presence of subepithelial osteocartilaginous focal lesions without any relation to tracheal rings, essentially localized in the lower two thirds of the trachea and the major bronchi. We report a new case of tracheobronchopathia osteochondrodysplasias in a 47-year-old man, localized at the lower half of the trachea, revealed by haemoptysis. The lesion was suspected by bronchoscopy and the diagnosis was made histologically, showing heterotopic bone formation. The evolution of this affection was marked by a good clinical tolerance with only a symptomatic treatment.