The coincidence of Infantile Hypertrophic Pyloric Stenosis (IHPS) and Esophageal Atresia (EA) with Tracheo-Esophageal Fistula (TEF) is rare. Although, vomiting and regurgitation in operated cases of EA and TEF are attributed to Gastroesophageal Reflux (GER) and the stricture of the anastomosis, it may be also associated with IHPS as well. We report a case of 3-hour-old female infant, who had EA and TEF operation and diagnosed to have IHPS at 9th week of age. Early diagnosis and treatment can prevent complications.