This is a case presentation of a 14-year-old boy with the radiological diagnosis of cholangiocarcinoma occluding the hepatic duct bifurcation. His only symptom at presentation was jaundice and further workup confirmed a mass at the porta hepatis. Surgical treatment resulted in a resection of the bifurcation tumor with a final pathological diagnosis of a carcinoid tumor of the hepatic duct bifurcation. To our knowledge, this is only the second case presented of a resected carcinoid tumor in adolescence. In this communique we present the above case and review of the world literature of biliary neuroendocrine tumors.