A 51-year-old woman with known dextrocardia presented with left-sided abdominal pain and symptoms consistent with biliary colic and cholelithiasis. Abdominal ultrasound confirmed the diagnosis of gallstones, as well as situs inversus with the liver and gallbladder on the left side and the spleen on the right. Laparoscopic cholecystectomy was performed without incident. The procedure was uncomplicated except for being the mirror image of that done with the gallbladder in the normal location. Cholelithiasis occurring with situs inversus is rare and may present a diagnostic problem. The extrahepatic anatomy of the biliary and venous system is the mirror image of the right sided liver. Historic and genetic aspects of situs inversus, as well as current theories regarding its etiology are presented. Situs inversus totalis does not appear to be a contraindication to laparoscopic treatment of cholelithiasis.