ObjectiveTo summary the treatment of pancreatic neuroendocrine neoplasms (pNENs). MethodsArticles relevant to pNENs at home and abroad were collected and reviewed. ResultsBecause of rare incidence and non-specific clinical syndromes of pNENs, clinician had no enough cognition about it. For pNENs, surgery was still the preferred option, combining other treatments included chemotherapy, somatostatin analogue, α-interferon, molecular targeted therapy, and peptide receptor radionuclide therapy (PRRT). ConclusionSurgery is still considered as the preferred option for controlling the associated biochemical syndromes and curtailing the malignant progression of pNENs.