The case of a 76 year old man with a progressive upper respiratory distress due to a chondroma of the trachea is reported which necessitated a segmental tracheal resection with end-to-end anastomosis and restoration of the initial tracheal lumen. This case report is illustrative of a rare benign disease (10 cases described till today) originating from the cartilaginous rings of the trachea. The tumor usually appears as an upper airway obstructing syndrome and endoscopy is essential for establishing the diagnosis. Laser resection can be recommended for the treatment of limited lesions, whereas tracheal resection with end-to-end anastomosis is recommended for more advanced chondroma because it is prone to local recurrence and potentially exposed to malignant transformation into chondrosarcoma.