Esophageal atresia (EA) with distal tracheosophageal fistula (TEF) develops hypertrophic pyloric stenosis (HPS) during the late postoperative period with a described prevalence of 1%-10%.The aim of this retrospective study is to show our experience in this association and to report two specific cases. Of 61 patients with EA treated in our institution, 2 developed HPS. Both cases underwent a Ramstedt extramucosal pyloromyotomy. Currently, patients are asymptomatic.Our frequency (3.3%) is similar to other publications, about 1%-10%. It is important to be aware of this association in a patient with a history of EA and recurrent vomiting in the late postoperative period. The etiology of this association should be investigated.