Aim To report the computed tomography (CT) findings of acute and complicated appendicitis in adults with incidental midgut malrotation. Materials and methods The medical records and CT studies of eight patients with appendicitis and incidental midgut malrotation who presented to two medical centres between 1998 and 2009 were reviewed. Results All patients presented with 1-5 days of acute abdominal pain, which was diffuse in two, left-sided in two, lower abdominal in two, and in the right lower quadrant in two patients. The inflamed appendix was right-sided in three, left-sided in three, and in the midline in two patients. Three cases were complicated by a peri-appendicular abscess, and one patient also had a small bowel obstruction. All patients had a complete non-rotation with right-sided duodenum and jejunum, and left-sided colon. All eight patients had an abnormal superior mesenteric artery-superior mesenteric vein (SMA/SMV) relationship and a dysplastic uncinate process of the pancreas. Urgent surgery was performed in six patients and the remaining two were treated conservatively. Conclusion Altered anatomy in malrotation affects the typical clinical and CT findings of acute appendicitis, therefore delaying diagnosis. When CT shows focal inflammation anywhere within the abdomen, along with an abnormal SMA/SMV relationship, the position of the caecum should be ascertained and acute appendicitis ruled out.