Maple Syrup Urine Disease (MSUD) is an inherited metabolic condition caused by a deficiency of the branched chain alpha-keto acid dehydrogenase enzyme complex that disrupts metabolism of the amino acids leucine, isoleucine, and valine. If left untreated, individuals with MSUD experience developmental delay, pancreatitis, and osteoporosis. Even with early and consistent treatment, individuals are at risk for metabolic crisis from acute illness or infection that can result in coma and death. Treatment requires a life-long low-protein diet and formula free of leucine, isoleucine, and valine.