Between 1960 and 1986, seven patients with intrahepatic cholangiocarcinoma and one patient with intrahepatic bile duct adenoma, related to hepatolithiasis, were seen among 112 cases of hepatolithiasis. Histopathologically, the tumors associated with hepatolithiasis arose from the periphery of the stone-containing bile duct, spread chiefly along the luminal surface, and invaded the ductal wall or periductal tissue. The tumors showed papillary to papillo-tubular proliferation and were diagnosed as the intraductal or periductal spreading type of cholangiocarcinoma. In addition, atypical epithelial hyperplasia was noted in the vicinity of the tumor area. These findings suggest that chronic relapsing cholangitis in patients with hepatolithiasis can induce progressive changes to atypical epithelial hyperplasia which may develop into cholangiocarcinoma.