Hepatoid adenocarcinoma (HAC) is a rare subtype of extrahepatic adenocarcinoma that is characterized by its morphological and functional similarities to hepatocellular carcinoma. We herein present a novel case of HAC arising from the extrahepatic bile duct in a 75-year-old Japanese woman with polysplenia syndrome. This is the second reported case of HAC arising from this site. The tumor induced jaundice and hemobilia. A total of four isolated intraductal polypoid masses of HAC were found. No recurrence was seen five months after surgery. Further reports of similar cases will be needed to clarify the clinical characteristics and the prognosis of this malignancy.