Extrahepatic duct duplication is an extremely rare congenital anomaly. Hilar cholangiocarcinoma with extrahepatic bile duct duplication was reported; however, intraductal papillary neoplasm of the bile duct (IPNB) with extrahepatic bile duct duplication has not been reported to the best of our knowledge. We report a rare case of IPNB with extrahepatic bile duct duplication of a 64-year-old female. The patient underwent extended right hepatectomy, and the results of a subsequence histopathological examination were consistent with an IPNB with extrahepatic bile duct duplication. We report this rare case with radiologic imaging findings and a brief review of the current literature.

Extrahepatic duct duplication is an extremely rare congenital anomaly. Hilar cholangiocarcinoma with extrahepatic bile duct duplication was reported; however, intraductal papillary neoplasm of the bile duct (IPNB) with extrahepatic bile duct duplication has not been reported to the best of our knowledge. We report a rare case of IPNB with extrahepatic bile duct duplication of a 64-year-old female. The patient underwent extended right hepatectomy, and the results of a subsequence histopathological examination were consistent with an IPNB with extrahepatic bile duct duplication. We report this rare case with radiologic imaging findings and a brief review of the current literature.