This study evaluated the nutritional status of children with three types of branched-chain amino acid disorders (MMA, PPA, and MSUD) in Riyadh City over two years. It found significant differences in growth between affected children and controls. Amino acid concentrations of isoleucine, valine, and glycine differed significantly between patient groups and controls. Ammonia levels were higher and albumin levels lower in patients compared to controls. The study concluded that some children with these disorders exhibited poor growth and characteristic amino acid profile differences.