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BACKGROUND Biliary neuroendocrine neoplasm (NEN) arising from the extrahepatic bile duct is rare, with an incidence of 0.2% among gastroentero-hepatopancreatobiliary NEN cases. Biliary neuroendocrine carcinoma (NEC) is an extremely rare high-grade malignancy that requires multidisciplinary treatment, including surgery, chemotherapy, and radiation. However, there have been only a few reports on the combined therapy for biliary NEC. CASE REPORT A 74-year-old man was referred to our hospital with a chief concern of obstructive jaundice. He was diagnosed with perihilar biliary NEC. The tumor primarily involved the confluence of the hepatic and left hepatic ducts. After 4 courses of systemic chemotherapy with cisplatin and etoposide (EP regimen), the tumor showed partial shrinkage. The patient underwent left and caudate hepatectomy with combined resection and reconstruction of the extrahepatic bile duct. Intraoperatively, strong adhesion between the right hepatic artery and the dorsal surface of the tumor was observed, requiring combined resection and reconstruction of the right hepatic artery. Postoperatively, the patient was treated with adjuvant chemotherapy (EP regimen) for 6 months. Ten months after surgery, he was diagnosed with multiple aggressive recurrences that were refractory to multimodal therapies. Eighteen months after the initial diagnosis and 12 months after surgery, he died of liver failure due to cholangitis. CONCLUSIONS This report presents the first case of perihilar biliary NEC that underwent perioperative chemotherapy and radical liver surgery. We believe that the introduction of perioperative chemotherapy is essential to achieve a better prognosis for perihilar biliary NEC, given its oncological malignancy and surgical invasiveness.