BackgroundAcute appendicitis is the most common cause of acute abdominal pain, requiring emergency surgery. Symptoms and signs of acute appendicitis usually occur in the right lower quadrant. However, approximately one-third of cases have pain unexcepted location due to its various anatomical locations. Acute appendicitis is a very rare cause of left lower quadrant pain; if it occurs, situs inversus (SI) and midgut malrotation (MM) are uncommon anatomic anomalies that complicate its diagnosis and management.Clinical presentationHere we present a 23-year-old Ethiopian male patient who presented with epigastric and left paraumbilical abdominal pain, fever, and vomiting of a day duration. On examination at admission, the patient had left lower quadrant tenderness. Later, with the help of imaging studies, the patient was diagnosed with left-side acute perforated appendicitis with intestinal nonrotation, and he was operated on and discharged improved after 6 days of hospital stay.ConclusionPhysicians should be aware that acute appendicitis in patients with intestinal mal-rotation may be present with left-side abdominal pain. Although it is extremely rare, acute appendicitis should always be considered in the differential diagnosis of left-side abdominal pain. An increase in awareness of this anatomical variant is essential for physicians.Supplementary InformationThe online version contains supplementary material available at 10.1186/s13256-023-03990-2.