Abstract: Situs inversus totalis (SIT) is a rare congenital condition, with an extremely low incidence. There is no difference between SIT individuals without onset of diseases and healthy counterparts. However, when SIT individuals suffer from diseases, the diagnosis and treatment are highly challenging due to insufficient understanding of SIT populations, especially for those complicated with end-stage liver disease and requiring liver transplantation. It is a huge challenge for surgeons whether SIT individuals serve as donors or recipients of liver transplantation. In this article, recent case reports related to liver transplantation in SIT patients were summarized, and the development, key procedures, clinical prognosis and postoperative complications of liver transplantation in SIT patients were reviewed.