Endocrine pancreatic tumors (EPTs) are uncommon, having an incidence of one per 100,000 people. They may appear as sporadic tumors or be associated with hereditary syndromes. EPTs are categorized as functioning or nonfunctioning tumors, based on the presence or absence of clinical syndromes. Among the former, insulinomas and gastrinomas are the most common. For the histopathological investigation of EPTs, chromogranin A and synaptophysin immunostainings are recommended. Measurement of circulating chromogranin A is also the cornerstone for the biochemical diagnosis of these tumors. Furthermore, specific hormones produced and released by the neoplastic cells can be identified by immunostaining and used for biochemical evaluation. To locate EPTs, both noninvasive (ultrasonography, computerized tomography, MRI and radionuclear imaging) and invasive techniques (arterial stimulation with venous sampling) can be used. Debulking procedures (surgery, radiofrequency ablation, embolization/chemoembolization and liver transplantation) and/or medical treatment (chemotherapy, biotherapy and peptide receptor radionuclide therapy) are the options available for the treatment of EPTs. Understanding the molecular events underlying the pathobiology of EPTs will aid the development of more accurate diagnostic/prognostic markers and give guidance for improved therapeutic modalities.