Unusual presentation of a low-grade intraductal papillary neoplasm of the bile duct

Summary Intraductal papillary neoplasm of the bile duct usually manifests as abdominal pain, jaundice, and cholangitis but rarely presents as hemobilia. In this article, we present the case of a 74-year-old man with a 2-year history of repeated hemobilia without a definite diagnosis. A cholangiogram revealed a left intrahepatic duct dilatation with a filling defect, and computed tomography revealed a hyperdense lesion in the left lateral liver segment, which was subsequently resected. Histopathological examination revealed focal low-grade intraductal papillary neoplasm of the bile duct and markedly dilated bile ducts with a ruptured blood vessel. The postoperative course was uneventful. Clinicians should comprehensively evaluate cases of unusual and recurrent gastrointestinal bleeding by considering this diagnosis.