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Neuroendocrine tumour of the appendix is a rare neoplasm discovered only incidentally. We hereby report an occurrence of this tumour in the appendix of a 27 years old female. There was an initial diagnosis of acute appendicitis based on a few days history of right abdominal quadrant pain, anorexia, vomiting and low-grade fever. The vermiform appendix obtained after an emergency appendectomy was large and had a firm yellowish mass, 1.6 cm in diameter. Histology revealed an appendiceal neuroendocrine tumour that had involved the mucosa, submucosa and part of the muscularis propria.