The present case report describes intraductal papillary mucinous neoplasms of the biliary tract (IPMN-BT), a rare neoplasm of the biliary tract that is described as the biliary counterpart of intraductal papillary mucinous neoplasms of the pancreas (IPMN-P). The importance of appropriate diagnosis and awareness of the clinical manifestations is highlighted. IPMN-BT has a more favorable prognosis and is easier to resect than other forms of intrahepatic cholangiocarcinomas; therefore, early and accurate diagnosis is required for planning of the best treatment strategies for this neoplasm.