Introduction : Pure mucinous cholangiocarcinoma is a very rare variant of intrahepatic cholangiocarcinoma.

Methods : An 83-year-old female was presented to our hospital with an epigastric pain. She had no liver disease. Contrast-enhanced abdominal computed tomography showed a dilatation of left hepatic duct containing both irregularly nodular enhancing components and hematomas. Endoscopic retrograde cholangiogram revealed a hemobilia of common bile duct. We initially diagnosed as an intraductal papillary neoplasm of the bile duct with hemobilia. Both left hemihepatectomy including middle hepatic vein and caudate resection was performed.

Results : Pathologic examination reported mucinous adenocarcinoma with directly invades into hepatic parenchyma and beyond the wall of bile duct. She was discharged without complication on postoperative day 9 and had no recurrence for 6 months.

Conclusions : Herein, we described a rare case of mucinous cholangiocarcinoma with hemobilia.