Carcinoid tumors of the extrahepatic bile ducts are extremely rare and account for only 0.2-2% of all gastrointestinal endocrine neoplasms. They are particularly difficult to diagnose preoperatively and distinguish from cholangiocarcinoma. We report the case of a 52-year-old patient with a carcinoid tumour of the common bile duct presenting with obstructive jaundice. Laboratory and imaging studies (US, CT, MRI) showed an obstructive lesion measuring 2 cm in diameter in the common bile duct. A biliary stent was inserted initially to decompress the bile ducts. Brushing was negative for malignant cells. With a suspicion of malignancy (confirmed intraoperatively), the patient underwent radical resection of the extrahepatic duct, portal lymphadenectomy and a Roux-en-Y hepaticojejunostomy. The pathology examination revealed a well-differentiated neuroendocrine tumour of the common hepatic duct. The tumour cells were immunopositive for chromogranin A and synoptophysin. There was no evidence of metastases in the nodes removed and negative surgical margins were achieved. The patient is disease-free after a follow up of 3 months, and levels of chromogranin A and neuron-specific enolase are normal. Local and systemic aggressiveness of these tumours is rare. Radical surgery offers the only chance of a cure and has to be considered whenever possible.