Background: Neuroendocrine tumor of the appendix is a rare neoplasm discovered only incidentally. They are mainly found in the histological examination of appendices operated due to acute appendicitis. The treatment is essentially surgical, and depending on the size of the lesion, it can range from a simple appendectomy to right hemicolectomy. Case presentation: In our work, we report the case of a neuroendocrine tumor of the appendix in a 15-year-old female patient admitted for symptoms of acute abdominal pain. She was diagnosed with appendicular peritonitis and admitted to the surgical unit. The anatomopathological examination of the vermiform appendix obtained after appendicectomy showed a histological aspect of a neuroendocrine tumor. Conclusion: We recommend that appendectomy specimens be routinely sent for pathological examination malignancy and NETs, may be missed without histopathological examination.