We present a case of 14-year girl with left-sided acute appendicitis who presented with lower abdominal pain. Midgut malrotation occurs at a rate of 1 in 500 live births. The condition is incidentally diagnosed during various radiological investigations done for other purposes. However, such patients may present with conditions like acute appendicitis, which poses a diagnostic dilemma if a high index of suspicion is not kept. The purpose of this case report is to increase awareness in the emergency physicians and young surgeons of this rare presentation; and the importance of radiological investigations in the diagnosis of left-sided appendicitis, to decrease morbidity and mortality.