Crohn’s disease is a chronic, idiopathic transmural inflammatory disease of the alimentary tract, which may be limited to the appendix in 0.2–1.8% of patients. Here, we report a male patient aged 24 years who presented with typical manifestations of acute appendicitis that proved histologically to be Crohn’s disease. On operation, an early phlegmon was found. The appendix was dissected and delivered. It was so large with very thick wall, with induration of the nearby cecal wall. Therefore, segmental right hemicolectomy was performed with iliocolic anastomosis. The specimen was sent for histopathologic examination, which demonstrated Crohn’s disease limited to the appendix, with other parts of the specimen free. We reviewed the literature on such clinical entity, its clinical significance, differential diagnosis, and best method of management. Actually, the disease needs to be differentiated from a long list of causes of appendicular granuloma as well as tumors and diverticulosis of the appendix. Histological confirmation is frequently required because of difficulty of conclusive diagnosis during surgery when the disease is limited to the appendix. The disease is usually benign and has indolent course than that developed elsewhere. Some authors debate the need for follow-up at all in those patients, believing that the appendectomy alone is curative in the majority of patients.