Primary neuroendocrine tumors of the extrahepatic bile ducts are extremely rare - up to date, only 77 cases have been reported in the literature, which represents between 0.2-2% of all gastrointestinal carcinoid tumors. The paper focuses on the case of a woman patient, aged 37 years, admitted to the Clinic with the diagnosis of obstructive jaundice, unaccompanied by pain and where imaging indicates a tumor in the third average of the common bile duct (CBD). The surgery involved the excision of CBD, lymphadenectomy and restoring the biliodigestive continuity of Roux-en-Y hepaticojejunostomy. The histopathological and immunohistochemical examinations revealed the presence of a well-differentiated neuroendocrine tumor of CBD. Based on the case report and literature, we attempted to accurately identify and relate this type of tumor to other varieties encountered in the extrahepatic bile ducts, pointing out elements of a positive diagnosis, differential diagnosis, histopathology and immunohistochemistry, and referring to the therapeutic attitude, evolutionary methods and prognosis.