Twenty cases of cholangiocarcinoma associated with hepatolithiasis were treated surgically. The incidence of cholangiocarcinoma associated with hepatolithiasis was 2.4%. Surgical procedures included common bile duct exploration after intraoperative choledochofiberoscopy in 20, with hepatic resection in nine. Overall survival was 8 months, with a range of 3-40 months. The hepatectomy group seemed to have a better prognosis. Accurate preoperative diagnosis of intrahepatic bile duct carcinoma associated with intrahepatic stones is difficult. All 20 patients in this series had surgery for recurrent cholangitis due to intrahepatic stones. From a retrospective review, it became apparent that early diagnosis can be obtained from the following: 1) detection of a hyperechoic mass in the liver parenchyma during abdominal ultrasonography, 2) a scintigraphic defect near the hilum, 3) filling defects or obliteration of intrahepatic ducts in ERCP or PTC, 4) gross appearance of the liver during surgery revealed a nodular tumor mass or an atrophic, fibrotic liver, and 5) intraoperative choledochoscopic findings which showed an intraluminal tumor or infiltrative lesion.