Situs inversus totalis is a rare anatomic variant in which there is a complete mirror image of the thoracic and abdominal viscera. The common association of situs inversus and biliary atresia has led to a variety of modifications of surgical techniques utilizing both living donor and deceased donor liver grafts, with mixed results in the pediatric liver transplant population. The use of a living donor liver graft in an adult with situs inversus has not yet been described. However, living donor liver transplantation (LDLT) has produced excellent results in the adult population, particularly in the cholestatic population, which may be disadvantaged by the model for end-stage liver disease system. This is the first report of a successful living donor right liver graft in an adult with end-stage liver disease secondary to primary sclerosing cholangitis and situs inversus totalis.