Abstract
Primary neuroendocrine tumors (NETs) of the bile duct are extremely rare and represent only 0.2-2% of all gastrointestinal NETs. Within the biliary system, the main bile duct is the most affected site. A 28-year-old man with a 6-month history of intermittent jaundice, pruritus, and choluria. MRCP, PET-CT and endoscopic ultrasound were performed. A well-differentiated neuroendocrine neoplasia was diagnosed. Complete resection of the main bile duct was performed with lymphadenectomy of the hepatic pedicle with Roux-en-Y hepaticojejunostomy, without complications. The patient had an adequate evolution and nowadays he’s disease-free. Primary neuroendocrine tumors of the bile duct are extremely rare. They may present clinically and radiologically similar to perihilar cholangiocarcinoma, which makes preoperative diagnosis difficult. Radical resection is indicated. Usually, they are well differentiated tumors, being the Ki-67 labeling index a reliable prognostic marker.