Adenocarcinoid tumour of the appendix is a rare entity characterised by the presence of a double component (neuroendocrine and glandular). It originates in the neuroendocrine cells of the appendicular mucosa. A preoperative diagnosis of a primary appendiceal tumour is uncommon and more so one suggesting an adenocarcinoid pathology. Optimal treatment is debated between a simple appendectomy and a more extensive resection, which occasionally includes hysterectomy and bilateral ovariectomy. Our aim is to report this rare entity and conduct a review of the literature on the different treatment options.