Neuroendocrine tumors include carcinoids and pancreatic endocrine tumors, which share a number of common features in their pathology, ability to cause clinical symptoms due to ectopic hormone and bioamine release, localization methods, and treatment. Although generally slow growing, a proportion demonstrate aggressive tumor growth. Therefore, in many cases treatment must be directed against both the tumor and the hormone-excess state. There have been significant recent advances into their molecular pathogenesis, natural history, and prognostic factors; clinical presentation defined by larger series analysis; localization methods; and the development of new treatments directed against the tumor and to control the hormone-excess state (including depot somatostatin analogues, interferon combinations, embolization, chemoembolization, radiotherapy with novel somatostatin analogues, and liver transplantation). Recent advances in each of these areas are briefly discussed.