IntroductionSitus inversus totalis (SIT) is a rare congenital abnormality, where there is transposition of both the thoracic and the abdominal organs, thus forming a left-right asymmetrical picture of the normal anatomical placement of the organs.1 It is kept under an umbrella term, heterotaxia, which encompasses all congenital disorders associated with malposition about the left-right axis.2 An important distinction is from simply situs inversus, where only some of the major abdominal and thoracic organs are reversed from their normal anatomical positions.3 SIT is even more of a rare condition, with an incidence of 1:10,000, and males being affected more than females (1.5:1).1 Due to this anatomical asymmetry, it may pose difficulties during diagnostic and therapeutic procedures.1,3Situs inversus totalis appears to be familial and laterality is established early on in the developmental process, during gastrulation. A protein, namely “Sonic Hedgehog” (Shh) is deemed to be responsible for the expression of two growth factors, Nodal and Lefty. If these proteins are released on the right side, the rotation of the heart occurs to the left side. However, if they are released on the left side, rotation occurs to the right. A gene named PITX2 has been identified, which controls the secretion of Shh and Nodal.4 The exact mechanism behind this is yet unknown, however, it has been speculated that the mutation in the PITX2 and Nodal gene is responsible and is also associated with other conditions like primary ciliary dyskinesia (PCD) and Kartagener syndrome.1,3,4,5Due to the unique transposition of the anatomical structures in SIT, the usual causes of abdominal pain may be a misdiagnosis. Similarly, these patients with cholelithiasis may have a presentation, with left upper quadrant pain, instead of the usual right, likely posing a diagnostic dilemma.6,7 Operative management in SIT patients also presents with technical and ergonomic challenges, especially for right-handed surgeons.8 The mirror image of the anatomy causes difficulty in the dissection of the Calot’s triangle, thus requiring appropriate tweaks to the normal preoperative and intraoperative setting.8 This report describes a case of a SIT patient with symptomatic cholelithiasis, managed by laparoscopic cholecystectomy.