Neuroendocrine tumors represent a group of neoplasias characterized by significant histopathological and biological heterogeneity. The basic study of the biological features of neuroendocrine tumors should allow the oncologist to identify those tumor subsets more sensitive to a particular medical treatment. For example, in metastatic or advanced disease, locoregional treatments, as well as radionuclide therapies, should be suggested only in selected cases. Although it has no significant effect on tumor growth, biotherapy with somatostatin analogs and/or interferon-alpha is recommended for either well-differentiated or functioning tumors. On the other hand, chemotherapy is effective in the treatment of those tumors characterized by a poor differentiation grade and a high proliferation rate. Novel therapies, new pharmacological formulations and more selective somatostatin analogs are now under clinical investigation for the treatment of neuroendocrine tumors.