Granulomatous inflammation of the appendix is uncommon. It can be caused by a variety of conditions, including systemic disorders such as Crohn's disease and sarcoidosis, and infections such as mycobacterium tuberculosis, yersinia pseudotuberculosis, parasites and fungi. Granulomatous appendicitis as an isolated pathological entity unassociated with systemic disease is rare. Isolated granulomatous inflammation of the appendix of unknown aetiology, otherwise known as idiopathic granulomatous appendicitis is extremely rare. Patients with this condition present with the typical signs and symptoms of acute appendicitis. We present a series of patients with isolated granulomatous inflammation of the appendix, and discuss the difficulties encountered in the management of this condition.