In recent decades, the appearance of inflammation near the appendix in patients with distal ulcerative colitis (UC) has been highlighted. Many epidemiological studies have confirmed the link between appendectomy and decreased incidence of UC. However, the clinical significance of appendiceal orifice inflammation (AOI) or peri-appendiceal red patch (PARP) as a "skip lesion" in UC has not been well elucidated. In this review, we summarized the literature regarding AOI/PARP and the role of this lesion in relation to UC. Since the appendiceal "skip area" in UC was first described in 1958, several reports using histologic examination of colectomy specimens and colonoscopy have been published. AOI/PARP has been more frequently associated with distal, mild UC than extensive, severe disease. Although it is still controversial, AOI/PARP seems to have little prognostic implication in the disease course of UC, including remission, relapse and proximal disease extension. However, some case reports have raised the possibility of a relationship between AOI/PARP and appendiceal neoplasms such as appendiceal cystadenoma and appendiceal adenocarcinoma. In addition, some investigators have treated UC patients who were resistant to conventional medical therapy with appendectomy and have reported inconsistent therapeutic effect. Further research may lead to the discovery of etiologic and pathogenic roles of appendiceal inflammation in UC.