Case 1. An 8-year-old boy with congenital cystic dilatation of the common bile duct had been treated medically for the erroneous diagnosis of autointoxication, since the age of 5 months, because of seizures of abdominal pain occurring 2 or 3 times a year. Dripinfusion cholangiograpy disclosed the pathological change. He was successfully treated with a choledocho-duodenostomy which was followed by a jejuno-gastrostomy in order to prevent retrograde biliary infection. Case 2. A 40-year-old woman had received a cholecystectomy at the age of 25 because of colic pain in the right hypochondrium and jaundice, but her symptoms persisted. The correct diagnosis of cystic dilatation of the intrahepatic and common bile ducts was made by percutaneous transhepatic cholangiography. A choledocho-jejunostomy using Roux-en-Y anastomosis was successfully performed. Case 3. A 2-yearold girl was strougly suspected of having a malignant intra-abdominal neoplasm because of an abdominal mass associated with jaundice. Pecutaneous transhepatic cholangiography revealed cystic dilatation of the intrahepatic and common bile ducts. She was cured by choledocho-jejunostomy using Roux-en-Y anastomosis. Conclusion The diagnosis of congenital dilatition of the biliary tract is sometimes difficult to make and can be confused with and treated as various other conditions. The most useful diagnostic tools are cholangiographies of all tyoes.