Appendicitis is a common disease in children. But left lower abdominal pain in acute appendicitis is a rare clinical feature. A 6 year-old-girl complained of left sided abdominal pain for 2 days. Past medical history was not contributory. Abdominal tenderness and guarding in left lower quadrant were noticed. Abdominal sonography and abdominal computed tomography scan demonstrated reversed position between superior mesenteric artery and vein, and a mass in the left lower quadrant abdomen suggesting appendicitis. Acute appendicitis in left lower quadrant, associated with intestinal malrotation, was found at laparatomy.