Although acute appendicitis is frequent, it is subject to common misconceptions. Furthermore, there is little good evidence to support some of our beliefs. This report reviews the role of the anatomic pathologist in diagnosis when acute appendicitis is suspected clinically and discusses what is known of its pathology. The conclusions that can be legitimately drawn from the literature are emphasized. A classification is proposed that incorporates intraluminal inflammation, acute mucosal inflammation, acute mucosal and submucosal inflammation, suppurative (phlegmonous) appendicitis, gangrenous appendicitis, and periappendicitis, and the significance of each of these diagnoses is discussed. The etiology and pathogenesis of acute appendicitis is reviewed. Contrary to popular belief, the best evidence indicates that obstruction is unlikely to be the primary cause, at least in the majority of cases. Ancillary techniques in the diagnosis of appendicitis, including laparoscopy and peritoneal aspiration cytology, are discussed.