Intraductal papillary neoplasm of the bile duct (IPNB) is a rare tumor arising from epithelia of bile duct, with a better prognosis compared with common cholangiocarcinoma. IPNB can occur anywhere along the biliary tree, and cause the dilation of the bile duct and acute cholangitis. Recent large case series of this peculiar disease in the far eastern countries showed greater incidences of hepatolithiasis and clonorchiasis compared with Western series. According to the immunochemistry features, IPNB can be classified into four subtypes, pancreaticobiliary, intestinal, gastric and oncocytic types. The pancreaticobiliary type is the most common and is usually associated with invasive lesions. Patients without distant metastasis can undergo surgical resection, surgical procedures include pancreaticoduodenectomy, hemihepatectomy, segmental liver resection and liver transplantation, and regional lymphectomy is necessary.