A variant of bile duct carcinoma, intraductal papillary neoplasm of the bile duct (IPNB) is a rare disease mainly found in Eastern Asia which encompasses a spectrum of intraductal papillary growth occurring anywhere along the intrahepatic and/or extrahepatic biliary tree that carries a high potential for malignancy. We report the case of a patient with episodes of recurrent cholangitis that was diagnosed with IPNB, our clinical and diagnostic approach, the radiographic and endoscopic findings, the interventions used, while discussing the therapeutic options.

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