Abstract: Neuroendocrine neoplasms (NENs) are a kind of rare tumors that originate from peptidergic neurons and neuroendocrine cells that show neuroendocrine differentiation and express neuroendocrine markers. NENs can occur in any part of the body; however, they are common in the lungs, stomach, intestines, and pancreas. National and international research data suggest a rise in the incidence of NENs. The results of epidemiological investigations from the United States have showed that, compared with other types of tumors, there was a more significant increase in the incidence of NENs. Based on existing evidence, in addition to national and international guidelines and consensus, the experts from China Anti-Cancer Association Committee of Neuroendocrine formulated its first guideline for the diagnosis and treatment of NENs to serve as a reference for clinical physicians.