Liver transplantation (LT) in an adult with situs inversus (SI) is extremely rare and requires precise pre-operative management. A 48-yr-old male with SI suffering from alcoholic liver cirrhosis underwent LT at our institution in March 2003. Pre-operatively, liver anatomy was determined by CT scan, three-dimensional liver reconstruction and angiography. LT was performed using the Belghiti technique with side-to-side cavo-caval anastomosis, transplanting a graft from a donor with normal anatomy. Post-operatively, the patient recovered without major complications, except an epileptic event because of a central pontine myelinolysis, and he was discharged on the 25th post-operative day. Three months after surgery, the T-drain placed intra-operatively into the donor bile duct was removed; transplant perfusion and function were stable with an actual follow-up period of 24 months. LT in patients with SI is feasible. Pre-operative imaging with three-dimensional reconstruction is a beneficial tool for operation planning in patients with rare anatomic variations.