Gallbladder neuroendocrine carcinoma (GB-NEC) is a rare clinical entity. In recent years, some advances have been made in terms of preoperative imaging diagnosis, survival prediction and treatment of GB-NEC. In clinical practice, the different patients' conditions and the efficacy of different treatment schemes should be taken into account to provide the most appropriate individualized treatment plan. Here, the authors review the clinical and basic research of GB-NEC in recent years, and summarize the pathogenesis, pathological classification, diagnosis, treatment progress and prognosis of GB-NEC based on the literature of case report of GB-NEC and other relevant data.