Congenital esophageal stenosis (CES) is associated with oesophageal atresia and tracheoesophageal fistula (EA-TEF). Tracheobronchial (cartilaginous) rests are typically found within the area of stenosis. The authors present a case report of EA-TEF associated with CES and present a modification of a novel technique to facilitate the management of CES. The impact of gastro-oesophageal reflux disease is discussed in this unique entity. Finally, the literature is reviewed for this unique entity.