Pancreatic neuroendocrine tumors (pNETs) are rare. Two objectives are essential in pNET treatment: control of symptoms of hormone-excess states and antitumor effect. Treatment of hormone-excess states is an emergency to avoid complications due to symptoms of hormone-excess state. Surgery is the only curative treatment. Others methods are used to control tumor mass. Guidelines of well-differentiated pNETs are different of poorly differentiated pNETs. Chemotherapy is established treatment of poorly differentiated pNETs. Systemic treatment (somatostatin analogs, chemotherapy and targeted therapy) and locoregional treatment (hepatic chemoembolization, radioembolization, radiofrequency ablation, peptide receptor radionuclide therapy) are used to treat primitive tumor and metastases, in patients with well-differenciated pNETs. All patients' files must be discussed in a specific multidisciplinary regional or national meeting, about pancreatic neuroendocrine tumors.