Maple syrup urine disease is an inborn error of amino acid catabolism characterized by a missing or defective branched-chain $\alpha$-keto acid dehydrogenase complex. Due to this deficiency, the oxidative decarboxylation of $\alpha$-ketoacids from the branched-chain amino acids (Ile, Val, and Leu) does not occur, leading to a build up of these $\alpha$-ketoacids and branched-chain amino acids in the blood and urine. The condition is associated with severe mental deficiency and failure to thrive, but it can be detected through screening in newborn (NB) blood samples and managed with a restricted diet.