Abstract: Neuroendocrine neoplasms, commonly deriving from neuroendocrine cells of gastrointestinal tract and pancreas, are remarkably heterogeneous. As for gastroentero-pancreatic neuroendocrine neoplasms, surgery is the first choice for treatment, whereas molecular targeted therapy provides a new treatment option for patients with local advancement or metastases. Drugs for molecular targeted therapy used clinically include somatostatin analogs, mammalian target of rapamyoin pathway inhibitors, tyrosinekinase inhibitors, immunotherapy and so on. Research findings from experiments and clinic trials will possibly provide new therapeutic methods for molecular targeted therapy in the future.