AbstractExtrahepatic bile duct Neuroendocrine Tumour (NET) is uncommon, and hence a preoperative diagnosis is rare. The definite diagnosis depends on post-operative histopathology examination and immunohistochemistry studies. Complete excision of the lesion is considered as a curative treatment. We report a case of 39 year old male who presented with obstructive jaundice and imaging suggested Cholangiocarcinoma involving the hepatic ducts. He underwent a right hepatectomy and cholecystectomy with hepatoduodenal lymphadenectomy. The histopathological examination revealed a Neuroendocrine Tumour.