Liver transplantation (OLT) in an adult with situs inversus (SI) is extremely rare and considered a contraindication because of the anatomic difficulties. A 45-year-old male patient with complete SI, suffering from progressive hepatic failure secondary to hepatolithiasis, obstructive jaundice, and liver cirrhosis, underwent transplantation in July 2004. Preoperatively the liver anatomy was determined by computed tomography scan, three-dimensional liver reconstruction, and angiography. OLT was performed using a modified piggyback technique, the donor right lobe was rotated 45 degrees to the left, making the donor left lobe point into the left paracolic sulcus and the donor right lobe in the recipient hepatic fossa. The donor's suprahepatic vena cava was sewn end to side to the recipient vena cava, and the infrahepatic vena cava oversewn. The patient recovered uneventfully, and transplant perfusion and function were stable at a follow-up of 40 months. The present study showed OLT in adult patients with SI to be feasible. The knowledge of exact anatomy, meticulous preoperative planning, and optimization of the recipients condition are essential.