The intraductal papillary neoplasm of the bile duct is a rare tumor considered one of the precursor lesions of cholangiocarcinoma. It is characterized by papillary growth within the bile duct lumen, occurring anywhere in the biliary tree. We report a case of a 70-year-old man who presented with a six-month history of weight loss and occasional choluria. An abdominal ultrasound showed a marked dilatation of the biliary tree with a polypoid intraluminal lesion. Computed tomography and magnetic resonance imaging confirmed the presence of these findings and did not reveal any secondary lesions. The patient underwent a cephalic duodenopancreatectomy yielding a diagnosis of intraductal papillary neoplasm with high-grade dysplasia.