Acute appendicitis is the most common cause of acute abdominal pain, requiring emergency surgery. Approximately one third of cases have pain unexcepted location due to its various anatomical location. Acute appendicitis is a very rare cause of left lower quadrant pain; if it occurs, a few congenital anomalies should be considered such as Situs Inversus totalis and Midgut Malrotation ( MM). MM is a rare congenital anomaly; it occurs due to error in process of rotation or fixation of intestines around the superior mesenteric vessels and it refers to nonrotation or incomplete rotation of intestines. Here we report a case who presented with left lower abdominal pain and was diagnosed with acute perforated appendicitis with intestinal nonrotation. Clinicians should be aware that intestinal nonrotation may be presented with left lower quadrant pain and complicated by acute appendicitis.