Congenital pyloric atresia is a rare disease, and only 38 cases have been reported in Japan. A 2-year-old girl was admitted to our hospital because of hypoproteinemia, anemia, and vomiting. She was diagnosed as blind loop syndrome with cholelithiasis due to the previous gastrojejunostomy which had been performed 2days after her birth for congenital pyloric atresia. Since atresia was fibrous-cord type, an end-to-end gastroduodenostomy after resection of the fibrous-cord with cholecystectomy was performed. After the operation, she has been asymptomatic. Congenital pyloric atreia is classified into 4types as follows; I. web or diaphragm, II. fibrous cord, III. segmental defects, IV. double membrane. It was suggested that an adequate operation should be selected for the congenital pyloric atresia according to its type of atresia in order to avoid the post-operative complications including blind loop syndrome and cholelithiasis.
