Neuroendocrine carcinomas mostly occur in the gastrointestinal tract, lung and pancreas. The gallbladder is an uncommon organ for a neuroendocrine carcinoma and it is difficult to diagnose before gallbladder surgery. Owing to its rare occurrence its natural course and treatment are not definitely established. A 71-year-old woman was hospitalized with intermittent abdominal pain and nausea. Abdominal computed tomography showed multiple masses of the gallbladder obstructing the biliary tract. From endoscopic ultrasonography we suspected carcinoma of the gallbladder that was infiltrating a common bile duct. A biopsy was taken using endoscopic retrograde cholangiopancreatography (ERCP) and the mass was diagnosed as a neuroendocrine carcinoma of the gallbladder. Here we report a case of a neuroendocrine carcinoma of the gallbladder confirmed by endoscopic biopsy, and provide a review of the literature.