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Appendiceal neuroendocrine neoplasms (ANENs) comprise rare tumors of the appendix, mainly affecting young populations and characterized by a rather favorable prognosis. The aim of this review was to summarize the current knowledge on these neoplasms, focusing on the management and follow-up of such patients, which still remain under debate. ANENs account for 0.16-2.3% of appendectomies and are usually diagnosed incidentally. The histopathological diagnosis includes the immunohistochemical profile of the tumor in regard to synaptophysin and chromogranin A, as well as the Ki-67 index. The surgical management of ANENs is either simple appendectomy or a more extensive oncological operation including right hemicolectomy. This depends on the stage and the presence of risk factors suggesting a more aggressive disease, such as the exact location, mesoappendiceal or lymphovascular …