Intraductal papillary neoplasm of the bile duct (IPNB) is a rare entity characterized by papillary growth within the bile duct lumen1, currently regarded as a biliary counterpart of intraductal papillary mucinous neoplasm of the pancreas. The most common clinical findings are abdominal pain, jaundice or cholangitis, although some patients are asyntomatic. The diagnosis requires careful assessment of imaging findings and cito-histological evaluation, with endoscopic ultrasound guided fine needle aspiration (EUS-FNA) playing a very important role. IPNB is a premalignant lesion which can evolve into invasive cholangiocarcinom. A radical treatment strategy should be planned accordingly.