Liver transplantation has become an accepted treatment for several metabolic liver diseases. With advances in organ transplantation and immunosuppressive strategies, survival rates following liver transplantation are generally excellent. When the primary metabolic defect is hepatic in origin, liver transplantation not only replaces the dysfunctional organ but also cures the underlying metabolic defect. For conditions in which the primary metabolic defect is extrahepatic, liver transplantation is usually performed for hepatic complications, although disease recurrence may occur. This article reviews common metabolic liver diseases treated with liver transplantation in the adult population.