According to the findings of operative cholangiography, 26 cases of congenital dilatation of biliary duct were divided into two groups; one with anomalous arrangement of pancreaticobiliary duct (anomalous group) and the other without such finding (unclarified group). Signs and symptoms in the unclarified group were those of typical choledochal cyst and obstructive cholangiopathia. On the other hand, the main symptom in the anomalous group was remittent abdominal pain, but mass and jaundice were not frequently seen. This condition was due to recurrent cholangitis caused by reflux of pancreatic juice. To minimize the risk of complications such as perforation of the bilialy duct, future development of malignancy and intrahepatic cholelithiasis, it is very important to recognize and to treat the anomalous arrangement of the pancreatico-bilialy duct in patients with choledochal cyst.