Objective To study the etiology, therapy and prevention of hepatolithiasis associated with intrahepatic cholangiocarcinoma(HLAIHCC). Methods A retrospectively study was made on clinical manifestation of 6 patients with HLAIHCC in two hospitals from March 1986 to June 1997. Results The main clinical manifestations of HLAIHCC were Charcot syndrome. All the places had stones where the cancer occurred. Pathologically, all of the HLAIHCCs were adenocarcinoma. Of them, 5 cases died within 1 year. Conclusions The intrahepatic cholingiocarcinoma is closely related to long term irratation of hepatolithisasis, inflammations and infections. Radical resection for HLAIHCC should be applied and that early term hepatic resection for intrahepatic stones might reduce the occurrence of intrahepatic cholangiocarcinoma.