For patients with MSUD to be candidates for liver transplantation, they need to be in good metabolic control, through dietary management of their branched chain amino acids (BCAA). In the perioperative period, continuous glucose infusion is necessary to prevent catabolism, sodium/water homeostasis maintained to avoid brain edema, and plasma amino acid analyses availability to monitor the BCAA levels. Studies report that after transplantation, these patients can consume a diet with no BCAA restrictions and no longer experience metabolic decompensation. There are no published studies reporting nutritional counseling during the transition to an unrestricted diet, nor the monitoring of growth and nutritional status to ensure that their dietary intake is appropriate.