A 67-year-old woman was pointed out dilatation of the intra-hepatic bile duct in the left lobe while she had been followed clinical course as a HBV career, and visited our hospital in April 2006. On admission, abdominal ultrasonography showed limited dilatation of the intrahepatic bile duct in the anterior lateral segment of liver and strong echo with the diameter of 5mm without acoustic shadow near the point of dilated bile duct. Abdominal CT scan revealed no calcification or mass which might have caused obstruction of the bile duct. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangio pancreatography demonstrated obstruction of the intrahepatic bile duct in the anterior lateral segment. We suspected the obstruction caused by cholangiocarcinoma, and performed left hepatic lobectomy. Intra-operative ultlasonography showed hepatolithiasis in a branch of the bile duct in the anterior lateral segment, but no mass was detected. The final pathological diagnosis was cholangiocarcinoma. Intraductal papillary growth of atypical cells was shown with interstitial microinvasion. The patient's postoperative course was uneventful. Intraductal papillary growth of neoplastic biliary epithelia (an intraductal papillary neoplasm of the bile duct [IPN-B]) resembling an intraductal papillary mucinous neoplasm of the pancreas is occasionally associated with hepatolithiasis.