Appendicitis is typically established when a patient presents with peri-umbilical abdominal pain associated with nausea or vomiting and subsequent radiation of the pain to the right iliac fossa. Rarely, left sided appendicitis has been reported in the literature and when it occurs invariably it is associated with a congenital anomaly of the abdomen either situs inversus or intestinal malrotation. We present here, a case of acute appendicitis in a patient with intestinal malrotation.