Abstract. Tracheal agenesis is an extremely rare, usually fatal, congenital anomaly presenting with respiratory distress and aphonia. We present a case of tracheoesophageal fistula, in which an endotracheal tube failed to be place; alternatively the infant was partially venitlated vian an endoesophageal tube. A delayed transfer of the infant and inability to perform an urgent tracheostomy led to the infant’s demise. Floyd described 3 different phenotypes of tracheal agenesis. The anomaly is thought to be due to errors in development occurring during the division of the foregut into the esophagus and the trachea. Affected neonates uniformly present in the immediate postnatal period with severe respiratory distress and aphonia. Endotracheal intubation is difficult, often with multiple failed attempts and inability to advance the tube below the vocal cords. Ventilation with a tube placed in the esophagus can temporarily sustain life in the presence of a fistula as in this case. The condition can occur in isolation or in association with malformations in organ systems other than the lungs, airway and esophagus. New approaches for reconstruction of the trachea have been reported and regenerative medicine may have a future role.