Hepatobiliary cystadenoma is a rare but distinct neoplasm with malignant potential. The differential diagnosis includes other cystic neoplasms, primary or metastatic, and nonneoplastic cysts including infective conditions. A 38-year-old woman was admitted because of abrupt epigastric pain. Abdominal imaging studies revealed about 15x10 cm sized multiloculated cystic lesion in left lobe of the liver. Both serum and cystic fluid level of CA 19-9 were raised above 2,400 U/mL. Laparotomy and left lobectomy were performed. A 15x10 cm sized cystic mass was noted in left lobe of the liver. About 1,800 mL of serous fluid was obtained from this multiloculated cystic mass. Microscopic findings revealed a cystadenoma lined by a single layer of columnar epithelium containing some intracellular mucin pools and hemorrhagic infarct without evidence of cellular atypism or mitosis. We report a case of intrahepatic biliary cystadenoma with markedly elevated CA 19-9 with the review of literatures.