Isolated type III tracheal agenesis without associated congenital anomalies is very rare. Therefore, infants with this type of isolated defects are potential candidates for surgical correction. We present three cases of tracheal agenesis (Floyd's type III) with no other major abnormalities and reviewed the literature. We also discussed the previously attempted surgical procedures with the hope that in future, with advances in surgical reconstruction, there may be a better solution for this lethal anomaly.