The clinical presentation of acute appendicitis and the idiopathic granulomatous variety are, as a rule, indistinguishable. Five patients with idiopathic granulomatous appendicitis, treated during past decade, had histological acute and chronic appendicitis abounding with granulomas. Investigations not disclosing a systemic or enteric granulomatous disease and the patients' long-term complete postoperative recovery support the diagnosis of idiopathic granulomatous appendicitis.