Objective：To discuss diagnosis and treatment of hepatolithiasis complicated with cholangiocarcinoma.
Methods：Fifty-four cases of hepatolithiasis complicated with cholangiocarcinoma from 1998 to 2009 treated in Zhongnan Hospital were collected and analysed.
Results：Cholangiocarcinoma occurred in 5.45% of patients with hepatolithiasis in our hospital group. The diagnosis was established preoperatively in 45 cases (83.3%). All of 54 cases underwent surgery. Among them, 21 cases (38.9%) of adenocarcinoma in bile duct underwent radically resected.  At followed up of 45 cases (83.3%), the average survival  time of patients that underwent radical resection was 23 months and that of patients who underwent drainage was 8.5 months.
Conclusions：The therapeutic effect of cholangiocarcinoma complicated with hepatolithiasis is poor and, when possible, radical resection of the tumor should be undertaken.