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Genetic obesity is responsible for up to 7% of severe childhood obesity. Although current Pediatric Endocrine Society guidelines recommend assessment of children with early-onset morbid obesity and hyperphagia for underlying genetic disorders, a vast majority of patients are not being appropriately screened for genetic obesity syndromes.With advances in genetic testing, more genetic causes of obesity are being identified. Treatments are likely to be individualized, depending on the cause of the obesity, and must be targeted at addressing the underlying cause. Investigational therapies include melanocortin-4 receptor antagonists, oxytocin and medications targeting the endocannabinoid system.Improved identification of patients with genetic obesity syndromes will lead to development of new treatments and personalized management of these diseases.