Congenital short trachea is a rare congenital anomaly in which the trachea is composed of reduced number of cartilage rings, which result in an abnormally high position of the carina and an abnormal course of the main bronchi. Hazards of congenital short trachea in infants and children include inadvertent bronchial intubation, because it causes bronchiostenosis, pulmonary interstitial emphysema, pneumomediastinum, pneumothorax, and ipsilateral atelectasis. Laryngeal cleft is a rare condition, as well. Symptoms range from mild stridor to massive aspiration and respiratory distress, depending on the severity of the cleft. Until now, a case with combination of these two rare congenital defects has not been reported. Herein, we report a 13 month-old boy who has congenital short trachea with laryngeal cleft.