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Maple Syrup Urine Disease (MSUD) is a rare inherited metabolic disorder manifested by the impaired catabolism of certain amino acids. This disorder results in the collection of toxic substances in the body, leading to a range of symptoms and complications. MSUD is categorized into different types dependent on the specific enzyme deficiencies involved in the disease. This review article aims to provide a comprehensive overview of the types of MSUD, along with their clinical features, genetic basis, diagnostic methods, and management strategies.