Background/aimThis study investigated the clinicopathological characteristics and treatment of appendix neuroendocrine neoplasms in appendectomy specimens of our center.Materials and methodsThe clinicopathological data, including age, sex, preoperative clinical manifestation, surgical method, and histopathological examination results of 11 patients with appendix neuroendocrine neoplasms confirmed by surgery and pathology between November 2005 and January 2023, were retrospectively analyzed.ResultsIn the histopathological examination of 7277 appendectomy specimens, 11 cases (0.2%) had appendix neuroendocrine neoplasms. Among the 11 patients, 8(72.7%) were males, and 3(27.3%) were females, with an average age of 48.1 years. All patients underwent emergency surgery. A total of 9 patients underwent open appendectomy, including 1 patient who underwent second-stage simple right hemicolectomy after an appendectomy, and two who underwent laparoscopic appendectomy. All 11 patients were followed up for a period of 1 to 17 years. All patients survived without any indication of tumor recurrence.ConclusionAppendiceal neuroendocrine neoplasms are low-grade malignant tumors originating from neuroendocrine cells. They are rarely seen in clinical practice and are often treated based on acute and chronic appendicitis symptoms. These tumors are challenging to diagnose before surgery due to the lack of specificity in clinical manifestations and auxiliary examinations. The diagnosis generally depends on postoperative pathology and immunohistochemistry. Despite the diagnostic challenges, these tumors have a favorable prognosis.