Situs inversus totalis is an uncommon congenital positional anomaly in which orientation of all asymmetric organs in the body are mirror image of normal morphology. The condition if undetected may pose a diagnostic problem of abdominal pathology. We present a case of situs inversus totalis with acute appendicitis in adult female who was previously unaware of her situs anomaly. A 35-year-old adult female presented with history of acute pain abdomen in left iliac region; clinically, she was diagnosed to be acute diverticulitis. Further investigation with abdominal computerized tomography (CT) and ultrasound imaging confirmed situs inversus with acute appendicitis. Patient underwent emergency laparoscopic appendectomy under general anesthesia; intraoperative electrocardiogram (ECG) monitoring was done with reverse lead placement.