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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