Esophageal atresia with tracheoesophageal fistula is a common form of congenital malformation of the esophagus, which was usually overlooked during the physical examination after delivery. The first report of esophageal atresia with tracheoesophageal fistula was done by Thomas-Gibson in 1696. We have performed primary repair by extrapleural approach through the right 4th intercostal space in 4 cases of congenital esophageal atresia with distal racheoesophageal fistula. End to end anastomosis was performed by Haight method in all cases. Postoperatively, two patients showed severe stenosis and one patient showed mild stenosis at the anastomotic site. Ballots dilatation was performed two cases with severe anastomotic stenosis.