Intraductal papillary cholangiocarcinoma (IPC) is a rare form of bile duct neoplasm with only 28 cases reported in the English literature to date. We report a rare case of an IPC arising in a liver containing many von Meyenberg complexes (VMC) in a 70-year-old woman. She presented with colicky right upper quadrant pain and nausea, mimicking biliary colic. Imaging studies showed a mass in the left lobe of the liver. A left hepatectomy, which included segments 2 and 3 was performed, and an ill-defined shiny polypoid nonhomogenous mass (8.2x2.9 cm) was identified within the lumen of a dilated bile duct without invading the liver parenchyma. Histologically, the tumor was composed of papillary fronds with fine fibrovascular cores lined by tall biliary columnar mucus secreting epithelium. Focally the cells were of the gastric foveolar type. The tumor extended into the peribiliary glands and merged with an adjacent large VMC. A possibility that the carcinoma may have originated in a VMC was considered. On follow-up after 12 months the patient is doing well and is without any disease or recurrence.