A55 -year-old woman with a 10×8mm protuberant gallbladder lesion visited our hospital. To determine possible malignancy, she underwent laparoscopic excision biopsy of the gallbladder, and pathological findings revealed an endocrine carcinoma in the gallbladder. Surgery involved extrahepatic bile duct resection, gallbladder bed resection, and hepatic portal lymph node resection. Recurrence in the peritoneal lymph node was observed in the early postoperative period. Chemotherapy was initiated with cisplatin and irinotecan, and continues 18 months post-operatively. Neuroendocrine carcinoma of the gallbladder is rare and is known to have a poor prognosis, with few confirmed treatments reported for this disease due to its rarity. Therefore, we report this case along with a review of the literature.