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The presence of a malrotation of the midgut in adults is identified in asymptomatic patients most commonly as an incidental finding during a workup for an unrelated disease. We report here a rare case of acute perforated appendicitis in a patient with nonrotation of the midgut. A 28-year-old man was referred to our hospital with lower abdominal pain. The radiological examination, including abdominal computed tomography, ultrasonography, an upper gastrointestinal series, and a barium enema, revealed acute perforated appendicitis accompanied by nonrotation of the midgut. Emergency surgery revealed a complicated appendix located in the middle area of the lower abdomen with a periappendiceal abscess and nonrotation of the midgut. An ileocecal resection was performed with no postoperative complication. In this case, the atypical position of the appendix led to confusion regarding the diagnosis and to a more invasive surgical intervention.