Domino liver transplantation is a procedure in which an explanted liver, from a patient with non-cirrhotic metabolic liver disease, is transplanted to a person with a different liver pathology which requires transplantation. A patient suffering from a monogenic metabolic liver disease, for which liver transplantation is a curative therapy, takes on the role of both recipient and donor. The recipient of his explanted liver may be a person whose life expectancy is considered to be shorter than the expected time for the onset of the metabolic disease caused by reception of such a liver. It can also be used as bridging therapy until definitive therapy or retransplantation. Domino liver transplantation was first performed in Portugal in 1995 on a person with familial amyloid polyneuropathy, while today such procedures have been performed in 66 hospitals around the world on more than 1,000 patients. Diagnoses of domino liver donors have expanded to maple syrup urine disease, primary hyperoxaluria, acute intermittent porphyria, homozygous familial hypercholesterolemia, propionic acidemia, hyperhomocysteinemia, and hemophilia A. The prognosis after a domino liver transplantation depends on a number of factors, the most important among them being an underlying liver disease. This paper offers a summary of the concept of domino transplantation, specific indications, risks and results to date.