During the period of 1979-1982, 10 cases of cholangiocarcinoma associated with hepatolithiasis were seen. We report the clinical features of 10 cases of cholangiocarcinoma in association with hepatolithiasis, along with detailed histopathology from the four resected specimens. Our observations suggest that whenever intrahepatic stones are encountered in patients over 50 years of age with a long history of recurrent cholangitis and intractable pain, further examination including echo-guided aspiration cytology, liver scanning, and peritoneoscopy should be performed to rule out a coexisting cholangiocarcinoma.