Abstract: Gastroenteropancreatic neuroendocrine neoplasms (GEP NENs) are exceedingly rare tumor, with an increasing incidence in recent years. According to the NANETS consensus guidelines for the diagnosis of neuroendocrine tumor, the algorithm for diagnosis of GEP NENs includes clinical syndrome suggestive of NENs, biochemical testing, genetic testing, tumor localization by imaging and tissue diagnosis. GEP NENs could be divided into functional versus non functional based on the clinical manifestations. Radical surgery is the standard first line therapy for limited stage tumors. However, two thirds of GEP NENs patients are inoperable for tumor metastasis at initial diagnosis. For these advance staged patients, multidisciplinary treatment is the best choice, which includes surgery, chemotherapy, biotherapy, molecular targeted therapy, somatostatin receptor targeted radionuclide therapy. Molecular targeted therapy may turn into a standard first line therapy for its good curative effect in recent studies.