ObjectiveTo summarize a patient diagnosed as Bismuth type Ⅲa hilar cholangiocarcinoma who unerwent the curative surgery combined with partial portal vein resection and reconstruction+hilar bile duct formation+Roux-en-Y choledochojejunostomy, meanwhile we reviewed the current status of surgical treatment of hilar cholangiocarcinoma at home and abroad.MethodsTo retrospectively summarized and analyzed the clinical data of one case of Bismuth type Ⅲa hilar cholangiocarcinoma. The preoperative total bilirubin of this patient was 346.8 μmol/L, and this patient underwent the curative surgery combined with partial portal vein resection and reconstruction+hilar bile duct formation+Roux-en-Y choledochojejunostomy after reducing jaundice by percutaneous transhepatic biliary drainage (PTBD). Then we retrieved domestic and foreign related literatures.ResultsOperative time of this patient was about 290 min and intraoperative bleeding was about 350 mL. No intraoperative blood transfusion occurred. The results of pathological examination showed middle-differentiatied adenocarcinoma of hilar bile duct with negative tumor margins and no regional lymph node metastasis (0/14). The postoperative recovery was uneventful with hospital stay time of 9 days and without any complication. The patient had been followed-up in the outpatient department for 3 years,and was generally in good condition. The evidence of recurrence or metastasis wasn’t found.ConclusionsPre-operative biliary drainage can improve the safety of operation and reduce the incidence of postoperative complications, extend liver resection for the patient with Bismuth type Ⅲa hilar cholangiocarcinoma, which can improve R0 resection rate and extend postoperative survival.