Situs inversus totalis (SIT) is a rare congenital anomaly characterized by complete transposition of abdominal and thoracic organs. Change in anatomical disposition of the organs not only influences the location of symptoms and signs arising from a diseased organ, but also imposes special demands on the diagnostic and surgical skills of the surgeon. We discuss two cases of SIT, outline how the diagnoses were made, and describe the pitfalls encountered during the surgery and how they were overcome. Although there is no current evidence supporting the increased incidence of cholelithiasis in patients with SIT, reports have shown that symptomatic gall bladder disease in these patients can be successfully and safely treated laparoscopically.