BACKGROUND Appendiceal neoplasms are rare and generally determined in appendectomy specimens for acute appendicitis. Depending on a tumor's histopathology and size, appendectomy or right hemicolectomy are the surgical treatment options. Adenocarcinomas, mucinous neoplasms, goblet cell carcinoids and neuroendocrine tumors are the types of the primary appendiceal neoplasm histopathology. In this study, we aimed to determine the incidence of appendiceal neoplasms in an acute appendicitis cohort. Also, histopathological distributions, demographic data, preoperative radiological diagnosis, and intraoperative findings were revealed for analysis, retrospectively. MATERIAL AND METHODS Between October 2011 and September 2017, 3554 appendectomies were performed for acute appendicitis in Bezmialem University Hospital, Istanbul Turkey. The medical records of these consecutive 3554 patients were evaluated retrospectively. After the histopathological analysis of the appendectomy specimens, a total of 28 patients were detected as having appendiceal neoplasm including appendiceal adenocarcinoma, low grade mucinous neoplasia, and appendiceal neuroendocrine tumors. RESULTS Appendiceal neoplasms were determined in 28 out of 3554 acute appendicitis patients with an incidence of 0.78%. According to the histopathological types, 3 of the cases (10.7%) were appendiceal adenocarcinoma, 8 of the cases (28.5%) were low grade mucinous neoplasia, and 17 of the cases (60.8%) were neuroendocrine tumors. The overall incidence of the appendiceal neuroendocrine tumors was 0.48%. CONCLUSIONS The information obtained from our study suggests that pathological examination of the specimen may not be necessary if there is no doubt according to preoperative radiological diagnosis and/or intraoperative findings of the surgeon.