Abstract
Background and Clinical Significance: Inherited metabolic disorders can result in fatal metabolic decompensation if not promptly recognized and treated. These conditions are common in Plain communities due to founder effects and the high prevalence of consanguinity. Case Presentation: We present the case of an adult Amish male with maple syrup urine disorder who sustained traumatic injuries and delayed metabolic intervention that contributed to a fatal outcome. Conclusions: This case highlights the critical need for increased awareness among emergency and adult care providers, especially in Plain communities, and emphasizes the importance of early multidisciplinary coordination and preparedness with metabolic resources to ensure timely, life-saving management in adult metabolic emergencies.