AbstractBackgroundAppendiceal neoplasms are rare and most present unexpectedly as acute appendicitis. The classification and management are confusing, and there are few substantial reports in the literature.MethodsA systematic literature review was performed to access relevant publications on the presentation, pathology and management of appendiceal tumours.ResultsAppendiceal tumours account for 0·4 to 1 per cent of all gastrointestinal tract malignancies and are found in 0·7 to 1·7 per cent of appendicectomy specimens. Carcinoid tumours are most common. Most are cured by simple appendicectomy if the tumour is less than 2 cm in size and does not involve the resection margin or mesoappendix. Epithelial tumours may present with, or in time develop, pseudomyxoma peritonei, the optimal management of which involves complete tumour resection and intraperitoneal chemotherapy, usually available only in specialized centres.ConclusionSuggested algorithms for the management of unexpected appendiceal tumours are provided. Recommendations are made for follow-up of patients with a perforated appendiceal epithelial tumour.