Benign biliary stricture is a challenging problem in hepatobiliary surgery. Benign biliary stricture is associated with major portal vein variation, which is not be found in literatures. A male patient with benign biliary stricture was admitted to the Chinese PLA General Hospital in March, 2010.The stricture was located in the hilar confluence with intrahepatic biliary dilation and hepatolithiasis. The result of computed tomography showed that the hilar biliary confluence was compressed by the left portal vein and right anterior portal vein. The patient was cured after receiving gallbladder interposition, choledocholithotomy and T tube drainage. We suggested that the benign hilar biliary stricture due to portal vein variation may be named as biliary nut-craker syndrome.