Since their first description in 1882, malignant neoplasms of the appendix still remain rare. Malignant carcinoid tumours are the most common accounting for 85% of all appendiceal neoplasms. Preoperative diagnosis is invariably difficult, and precise treatment protocols for these neoplasms remain unclear. We reviewed our experience and searched published evidence to produce management guidelines. A retrospective review of all malignant carcinoid tumours diagnosed in our hospital between April 1994 and December 2003 was performed. Patient demographics, operative details, histological types and clinical outcomes were retrieved from case notes supplemented by a questionnaire to the patient's General Practitioner. A literature search was then performed. Nine patients were identified with classical carcinoid tumours (CCT); (M = 3 and F = 6, mean age: 43, range 14-81) and two patients with goblet-cell morphology (F = 2, age 46 and 76). Mean follow-up was 63 months (range 1-125 months). Nine patients were alive at the end of follow-up. Appendicectomy was performed for acute appendicitis. Other appendiceal pathologies were identified following hemicolectomy and oophorectomy. CCT are the most common tumours and have the better prognosis. From our experience and subsequent review of the literature, we recommend right hemicolectomy as the treatment of choice for malignant carcinoid tumours. However, small CCTs less than 2 cm in diameter at the tip of the appendix, with a low proliferative index, without angiolymphatic or mesoappendiceal extension can be treated by appendicectomy. Following oncological assessment, further adjuvant therapy should be considered for patients with advanced disease.