A patient with left upper quadrant appendicitis correctly diagnosed preoperatively is presented and the four cases previously reported are reviewed. The rarity of left-sided appendicitis coupled with the frequent referral of pain to the contralateral side often leads to an erroneous diagnosis. The physical examination is of limited value unless dextrocardia is noted. This case demonstrates the value and safety of a preoperative barium enema in localization of the appendix. The intraoperative management of unsuspected ectopic appendicitis is detailed. The use of a secondary midline incision is recommended if the cecum is not located on the right. Prophylactic appendectomy and fixation of the intestines appear to be warranted if asymptomatic nonrotation is discovered, because of the high incidence of appendiceal perforation as well as later partial small bowel obstruction due to intermittent volvulus.