Purpose of review Neuroendocrine tumors of the pancreas are a small subgroup of tumors characterized by a variety of biological behaviors. Recent changes in their classification should help better define the prognosis of this diverse group of tumors. With recent advances in diagnosis and staging, the treatment options for all neuroendocrine tumors have evolved. Presented here is a review,of the current-day knowledge for neuroendocrine tumors of the pancreas. Recent findings A consensus by leading experts in the neurendocrine tumors field has proposed an algorithm for the diagnosis, treatment and follow-up of these rare tumors. Surgical resection remains the first-line therapy. Alternative forms (if cytoreduction such as radiofrequency ablation,ana embolization, have increased the ability of the surgeon to debulk these tumors, resulting in improved survival and better palliation. Contrary to adenocarcinoma of the pancreas, hormonal and biotherapy offer unique treatment strategies for these rare tumors. Very recent developments utilizing radionuclide therapy hold promise for not only palliation, but may prove to be a beneficial form of adjuvant therapy. Summary Presented here is a summary of the recent literature on the diagnosis and treatment of neuroendocrine tumors of the pancreas.