Tracheal agenesis is an extremely rare congenital anomaly with fatal consequences. We report a case of tracheal agenesis in a newborn infant who presented with no self-respiration and cyanosis that was confirmed by the use of 64-slice multidetector CT. Coronal and sagittal multiplanar reconstruction images clearly delineated the aberrant anatomy of tracheal agenesis and demonstrated the presence of a tracheoesophageal fistula.