Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 10 3 2025 12 09 138 141 Aysan Nozheh Department of Pathology, Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran. Seyed Alireza Hosseini Department of Surgery, Hakim Children’s Hospital, Tehran University of Medical Sciences, Tehran, Iran. Maryam Sotoudeh Anvari Department of Pathology, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran. 2025 09 30 2025 10 04 Neuroendocrine tumors (NETs) are rare neoplasms that arise from neuroendocrine cells, with a significantly lower incidence in children compared to adults. In the pediatric population, appendiceal NETs are particularly notable, accounting for approximately 87.5% of cases, while the overall incidence of NETs in children is estimated at about 1.14 to 5.4 cases per million, compared to around 5.25 per 100,000 in adults. This illustrates the rarity of NETs in children and the difficulties in diagnosing them. These tumors often display symptoms similar to acute appendicitis, leading to their accidental detection during appendectomy. The vague clinical features of appendiceal NETs make preoperative diagnosis and management challenging. In children, these tumors are usually well-differentiated and have a positive outlook; however, the absence of specific pediatric guidelines has led to an excessive reliance on adult treatment protocols, which may be overly aggressive. This case study recounts the experience of a 10-year-old girl who exhibited symptoms suggestive of acute perforating appendicitis. Surgical intervention revealed a neuroendocrine tumor within the mid-portion of the appendix lumen, highlighting the crucial role of histopathological examination in such instances. The findings underscore the need for increased awareness among clinicians about the potential for neuroendocrine tumors to imitate common surgical conditions like appendicitis. Additionally, this case emphasizes the importance of developing pediatric-specific management guidelines to enhance care and minimize unnecessary surgical interventions for this distinct patient group. https://crcp.tums.ac.ir/index.php/crcp/article/view/1104 https://crcp.tums.ac.ir/index.php/crcp/article/download/1104/734