A 65-year-old female patient came to our hospital on January 11th, 2018 for repeated pain in the upper abdomen for 4 months and skin sclera for 1 week. At the time of admission, the patient's skin and sclera turned yellow, mild abdominal pain, no fever and abdominal tenderness. Laboratory examination showed obstructive jaundice changes accompanied by an increase in Carbohydrate antigen 19-9 (CA19-9). The patient underwent a contrast-enhanced computed tomography (CT) and magnetic resonance imaging examination in our hospital. The results showed that soft tissue-like density lesions were seen in the bile duct of the hilar region, and the lesions involved the upper segment of the common bile duct. Enhanced scanning lesions showed mild enhancement. Considering Bismuth-Corlette II type of hilar cholangiocarcinoma, intrahepatic bile duct dilatation was evident above the tumor site. The structure of the portal hepatic hilum area was not clear, considering the possibility of tumor invasion. Multiple lymph nodes were swollen after the retroperitoneum. Magnetic Resonance Cholangiopancreatography (MRCP) showed hepatic hilar bile duct truncation, and the intrahepatic and extrahepatic bile ducts were significantly dilated above the obstruction. No significant expansion of the main pancreatic duct. No intrahepatic or distant metastases were found. After detailed preoperative discussion and preparation, resection of the extrahepatic bile ducts with concomitant radical lymphadenectomy and Roux-en-Y cholangiojejunostomy was performed. During the operation, hepatic cholestasis was observed, and the hilar bile duct had a mass of 2 cm × 3 cm × 2 cm. The portal vein has not been invaded by the tumor. No perioperative complications occurred and the patient was discharged on the 12th day after surgery. According to the eighth edition of the AJCC tumor staging [], this patient was stage 1 (pT1N0M0). After she discharged from hospital, she was followed-up by telephone once a month, and she came to hospital for liver function test, CA19-9 and abdomen contrast-enhanced computed tomography. Until now, no abdominal pain, no skin or scleral yellowing and other abnormalities, no signs of recurrence and metastasis. A 2 × 1.8 × 1 cm mass was seen in the hilar bile duct and invaded the entire bile duct wall. At light microscope, two tumor components of adenocarcinoma and neuroendocrine cancer could be seen as collision type. Adenocarcinoma cells were columnar, cubic, and nuclear division were rare. Neuroendocrine cancer tumor tissues were solid, flaky, trabecular or organ-like. The cells were small and round, and the cytoplasm were sparse. There were abundant sinusoids in the middle, and the Ki-67 index was 70%. Neurological invasion was occurred, no intravascular tumor thrombus was seen, and no involvement of cancer in the bile duct margin. No lymph node metastasis. Immunohistochemistry and special staining: CK7 cholangiocarcinoma ( +), CK19 ( +), CD34 vascular ( +), NSE nerve ( +), Ki67 (+, 70%), CgA, Syn neuroendocrine carcinoma ( +), p53 (cholangiocarcinoma 3 +, neuroendocrine carcinoma 2 +), VG ( +).