Pancreatic neuroendocrine tumors are rare spleen tumors originated from the endocrine part of the pancreas with low incidence and malignant tendency.Therefore,early diagnosis and effective treatment are the key to improve the survival rate of patients.At present,the diagnosis of pancreatic neuroendocrine tumors is mainly based on laboratory examination,imaging examination and pathological examination.Surgery is the treatment of choice for patients with pancreatic neuroendocrine tumors,other treatments include chemotherapy,peptide receptor radioisotope therapy,molecular targeted therapies and somatostatin analogues.This paper combined with new basic research and clinical trials,the diagnosis and treatment of pancreatic neuroendocrine tumors are reviewed.