Abstract: Objective To analyze the clinical features of cholangiolithiasis with cavernous transformation of the portal vein ( CTPV) and to investigate the surgical treatment of this disease. Methods A retrospective analysis was performed on the clinical data of 18 patients with cholangiolithiasis with CTPV who were admitted to our hospital from January 2009 to December 2012; the clinical data included treatment method, surgical approach, time of operation, blood loss during operation, complications, and stone clearance rate. Results No death was seen in these cases. Eight cases underwent endoscopic retrograde cholangiopancreatography, and 3 cases underwent stone removal through the porta hepatis. The time of operation was 2. 9- 5. 4 h ( mean, 3. 8 h) . The blood loss during operation was 150- 2630 ml ( mean, 430 ml) . The rate of residual stones was 11%. Conclusion For patients with cholangiolithiasis who have a history of chronic liver diseases and who have undergone biliary tract surgery several times, especially those with recurrent biliary tract infection, examination should be carried out to detect CTPV, and individualized therapy should be determined before operation according to imaging data, liver function, and general condition. It is better to remove all stones using a choledochoscope during operation, and ultrasound- assisted hepalobectomy can be performed if necessary to remove intrahepatic stones.