ObjectiveTo investigate the clinical manifesfations and diagnostic and therapeutic features of cholangiocarcinoma associated with hepatolithiasis.Methods The clinical data, the diagnotic and therapeutic featares of 54 cases of cholangiocacinoma associated with hepatolithiasis were retrospectively analyzed.Results The occurrence rate of hepatolithiasis concomitant with hepatocholangiocarcinoma was 11.8%.Due to a lack of specific clinical manifestations, the preoperative diagnosis of this condition was difficult. In this series, the correct diagnotic rate of hepatocholangiocarcinoma before operation was only 11.1%. The radical resection rate was 51.8%. Radical resection of the tumor had a better prognosis than that of nonresection of tumor.ConclusionsPatients with longterm recurrent hepatolithiasis tended to have associated cholangiocarcinoma. Early diagnosis of the disease was difficult, and the treatment results and prognosis were poor. Therefore, patients with hepatolithiasis, espesially those with recurrent attacks, should undergo operation early. In cases diagnosed as hepatic cholangioearcinoma at operation, a radical resection should be performed, if possible, and a favorable outcome may be attained.