Objectives: The aim of this retrospective bicentric study is to review the incidence and treatment outcomes for children with appendiceal NETs. Patients and methods: Between January 1, 2009, and November 1, 2023, a sum of 6285 appendectomies have been conducted across two major Croatian pediatric centers. Following a detailed retrospective examination of patient records and their histopathological findings, a total of 31 children (0.49%) have been identified with appendiceal NETs. Consequently, these cases were analyzed further. The main objective of the study was to determine the incidence and subsequent treatment outcomes of the pediatric patients that have been diagnosed with appendicaeal NETs. The secondary objectives consisted of assessing the demographic, laboratory and clinical profiles of our patients, as well as the histopathological characteristics of the tumors. Results: The total incidence of appendiceal NETs remained consistent throughout the study period, only with slight variations. The patients median age was 14 years (Interquartile range, IQR: 12–16), with a higher prevalence among females (64.5%). Most of the patients (96.8%) have presented acute symptoms of abdominal pain and also went through appendectomies due to suspected acute appendicitis. Histopathological analysis confirmed acute appendicitises in 18 cases (58%). None of the NETs in the appendix were detected before surgery. Among those with confirmed acute appendicitis, the majority (14 patients, 77.8%) had non-perforated acute appendicitis. The tumors were predominantly located at the tip of the appendix (18 cases, 58.1%), with most measuring less than 1 cm in diameter (21 cases, 67.7%). The mitotic count was low in 25 patients (80.6%), and the Ki-67 proliferation index was also low in 23 patients (74.2%), leading to most tumors being classified as NET G1 (25 cases, 80.6%), with NET G2 and G3 being found in four (12.9%) and two (6.5%) patients. All of our patients were treated solely with appendectomy with a median follow-up period of 54 months (IQR: 24–95). Conclusion: The occurrence of appendiceal NETs is exceedingly rare in the pediatric population. These tumors are most commonly found in adolescents, with a higher incidence in females. The majority of the tumors size up to less than 1 cm, they are usually located at the appendix's tip. Appendiceal NETs are usually linked to non-perforated appendicitis. Appendectomies were the definitive treatment in all cases, with no need for more extensive surgery in this cohort.