This is a case report of cholangiectasis with cholangiocarcinoma in a 37-year-old female. Both computed tomography (CT) and endoscopic retrograde cholangio-pancreatography (ERCP) demonstrated gallstone, and intrahepatic bile duct dilatation with the stone. The diagnosis was intrahepatic cholangiectasis without common bile duct-dilatation. Hepatectomy of segment 3 with resection of the extrahepatic bile duct and reconstruction of the biliary tract was performed. Upon pathological examination, an early stage of cholangiocarcinoma was pointed out within the small range of common bile duct without dilatation or stone. Cholangiocarcinoma with cholangiectasis has been reported to be induced by counter flow of pancreas and intestinal juice because of the pancreaticobiliary maljunction. So we usually choose a diversion operation as our tactics to get rid of the counter flow of those digestive juices. Despite the above-mentioned tendency, this is a very rare case of carcinogenesis in common bile duct without dilatation.