Three cases of congenital pyloric atresia are described and a review of 100 cases reported in the literature is presented. Two of the cases were the membranous type. For these cases, excision or incision of the diaphragm with pyloroplasty was performed. The first patient was alive following the second operation of gastroduodenostomy for stenosis after excision. The second premature patient (1,380g of birth weight) died of sepsis on 58th postoperative day. The type of the third case was the fibrous cord, which required a resection of pylorus with end-to-end gastroduodenostomy. The patient did well postoperatively. With a review of literature, incidence, etiology, type of atresia, sex ratio, polyhydramios, birth weight, consanquinity, familial occurrence, symptoms, diagnosis, treatment and mortality rate were discussed. The treatment should be surgical and the operative procedure is variable due to the type of atresia. Excision or incision of the diaphragm with pyloroplasty and gastroduodenostomy is the procedure of choice. Gastrojejunostomy should be avoided because of its high mortality rate.
