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Situs inversus totalis (SIT) is a rare entity characterized by mirror-image reversal of the viscera. The coexistence of SIT and Leriche syndrome has been rarely described. The authors describe the surgical management of juxtarenal aortic occlusion in a patient with SIT which was successfully managed with open aortic endarterectomy and distal bypass. The malposed organs are at a greater risk of operative injury, but surgical management is feasible with proper determination of the anomalies on preoperative imaging.