We herein present a rare case of acute appendicitis with intestinal malrotation. Coronal images of contrast-enhanced computed tomography (CT) revealed the small intestine on the right side and the large intestine on the left side, thus indicating intestinal malrotation (non-rotation type). In addition, an enhanced, tubular, fluid-filled structure was detected attached to the cecum, which was located superior to the urinary bladder, suggesting acute appendicitis. The present study shows that coronal CT images provide important information for the diagnosis and treatment of acute appendicitis in patients with intestinal malrotation.