Neuroendocrine tumors (NET), or more generally neuroendocrine neoplasms (NEN), represent a very heterogeneous group of rare tumors with varying location which are only defined by their endocrine biology and secretion of synaptophysin and chromogranin A. They originate from mesoderm-derived stem cells. In the last few years, the incidence and prevalence of NEN have been steadily increasing. Classification is based on the affected organ, the proliferation rate and presence or absence of hormone production with typical symptoms. Diagnosis and treatment of these tumors is therefore very specific and requires an interdisciplinary approach. Treatment options include endoscopic or surgical resection, drug therapy for control of symptoms and proliferation, locoregional therapy and radionuclide therapy. Guidelines with algorithms for diagnostic workup and treatment are constantly updated.