Background: Neuroendocrine Neoplasms (NEN) represent 60% of all appendicular tumours. This type of cancer is predominantly benign.  In this study, appendicular NEN tumours in children were investigated.
Materials and Methods: This retrospective study was conducted on 540 patients underwent emergency appendectomy for the treatment of clinically suspected appendicitis at the department of pediatric surgery in Hedi Chaker hospital in Sfax, Tunisia.  This study was performed between January 2013 and December 2016. Data on appendicular NEN demographic, preoperative diagnoses, investigations, operative findings, pathological reports, and follow up were reviewed and analysed.
Results: Out of 540 patients, there were 309 males (57.3%) and 231 females (42.7%).  The Mean age of the patients was 9.23 ± 2.78 years. One hundred and thirty seven patients (25.4%) had laparoscopic appendectomy and 74.6% were operated by the traditional open approach. The diagnosis of appendicular NEN tumours was histologically confirmed in 4 appendectomy specimens. Clinically, all patients presented with acute appendicitis with raised inflammatory markers and positive ultrasound for appendicitis. The mean of tumour size was 1 cm. Complete resection was successfully achieved in all patients. The mean of follow-up was 3 years. 
Conclusion: Appendicular NEN tumours were diagnosed postoperatively with a histological examination. It seems that surgical management with simple appendectomy seems to be curative for the majority of cases especially with tumours less than 2 cm. according to this study, the prevalence of appendicular NEN tumours is low between all gastrointestinal tumors.