Appendiceal neuroendocrine neoplasms are uncommon, mostly discovered coincidentally during appendectomy. They usually show a benign clinical course and appendectomy alone is curative. However, some cases may harbor malignant potential; therefore, additional/prophylactic operations, such as right hemicolectomy, are offered. Current international guidelines are based on heterogeneous and retrospective series. Thus, there is lack of robust evidence, mainly in terms of accurate factors, that could identify patients at risk, requiring more extensive surgical treatment. In this article, we highlight controversies in the epidemiology, workup assessment, and management algorithms of appendiceal neuroendocrine neoplasms, but also to explore future developments and advances.