Crohn's disease is a condition of chronic inflammation involving potentially any location in alimentary tract, but it is worldwide the rare when primary lesion with its extent is confined to the appendix alone. Because clinical manifestations of the Crohn's disease of the appendix dose not differ from those of acute appendicitis, it is difficult to diagnose preoperatively or intraoperatively, so it is usually diagnosed by postoperative pathologic examination. Because Crohn's disease confined to the appendix has favorable prognosis than typical Crohn's disease, it is considered a separate disease entity from Crohn's disease, and a new diagnostic term-"idiopathic granulomatous appendicitis"-has gained wide acceptance in recent years. We experienced a case of Crohn's disease confined to the appendix or idiopathic granulomatous appendicitis and review, we reported the care with review of the literatures. (Korean J Gastrointest Endosc 2005;31:4952)