A gallbladder neuroendocrine neoplasm (GB-NEN) is a unique heterogeneous neoplasm, originating from neuroendocrine cells and able to secrete peptides as its neurotransmitter. Among all the neuroendocrine tumors (NETs), the GB-NEN prevalence is 0.5%, which accounts for ~2.1% of all gallbladder tumors. The current study aimed to present an up-to-date review of the clinical features, diagnosis and treatment of NETs of the gallbladder. A systematic literature search was performed in order to identify all relevant studies published. Thus far, the etiology and pathogenesis of GB-NEN remains unclear. Moreover, an ambiguous clinical manifestation exists as observed during laboratory examinations. As GB-NEN is a rare gallbladder lesion and is seldom seen in clinical practice, there is a limited availability of review reports, and it is therefore often considered only as a case study. It is difficult to distinguish GB-NEN from other gallbladder diseases using imaging diagnostic techniques. Although chemotherapy treatment provides prolonged progression-free survival, surgery is considered the best option. Thus, to overcome the inherent risks or shortfalls of traditional surgery; early detection, diagnosis and treatment of GB-NEN are required to improve patient longevity.