Abstract: Pancreatic neuroendocrine tumors (PNETs) have an annual incidence of approximately 1 per 100, 000 individuals and most often occur in adults aged 40 years to 60 years. PNETs, also called islet cell tumors, originate from the Langerhans cells in the pancreatic islet. Based on the presence or absence of specific hormone-related symptoms, PNETs are classified as either functional or non-functional. Depending on their clinicopathological characteristics, these tumors can also be classified as G1, G2, or G3 in the World Health Organization classification and grading system. The American Joint Committee on Cancer and European Neuroendocrine Tumor Society has also introduced the TNM staging classification for PNETs. New technologies, including molecular markers and medical imaging, have been used to diagnose PNET and have significantly improved the diagnostic accuracy. As for the treatment of PNET, surgery may be the only option. Different modes of surgery can be used depending on the tumor type, location, and size. Clinical application of traditional chemotherapeutic agents is limited because of their toxicity. However, with the increased understanding of PNET pathogenesis, therapeutic agents such as somatostatin analogs, angiogenesis inhibitors, and mTOR inhibitors have already been used in clinical practice and have brought hope to patients.