The Multi-Organ Transplant Committee (MOTx) of the International Pediatric Transplant Association (IPTA) presents a case of transplantation for a patient with methylmalonic acidemia (MMA), an autosomal recessive disorder disrupting normal amino acid metabolism and leading to hyperammonemia. Treatment with liver transplant alone and combined liver and kidney transplantation have led to increased survival rates in children. Discussion will center on treatment decisions leading to liver transplant alone vs. dual liver/kidney listing, challenges of pediatric organ transplant listing for MMA, and outcomes.