Intestinal malrotation and situs inversus can have important repercussions if acute abdominal pain develops. Intraabdominal structures can have inverted position and thus may easily mislead the surgeon during physical examination. Fortunately, radiological exams have improved the preoperative diagnosis of these patients. However, in difficult cases when an underlying surgical disease is suspected, laparoscopy remains the gold standard in order to diagnose and treat if possible the suspected disorder. We present a case of acute left-side appendicitis in a child with unknown congenital intestinal malrotation. In addition, this case stressed the value of laparoscopy in daily practice to evaluate patients with atypical abdominal pain.

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