Roshni Vara, Charles Turner, Helen Mundy, Nigel D. Heaton, Mohammed Rela, Giorgina Mieli‐Vergani, Mike Champion, Nedim Hadzic – 25 January 2011 – Propionic acidemia (PA) is a rare inherited disorder of branched chain amino acid metabolism; despite improvements in conventional medical management, the long‐term outcome remains disappointing. Liver transplantation (LT) has been proposed to minimize the risk of further metabolic decompensations and to improve the quality of life. We performed a retrospective review of all children with PA who underwent LT between 1987 and 2008.