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OBJECTIVE To verify the occurrence of overweight in children and adolescents with phenylketonuria and to identify possible causal factors. DATA SOURCES A systematic review was performed in the SciELO, PubMed and VHL databases using the descriptors "Phenylketonurias", "Overweight", "Child" and "Adolescent". Original articles conducted with children and adolescents, published between 2008 and 2018 in Portuguese, English or Spanish languages were included. DATA SYNTHESIS A total of 16 articles were identified and, after screening procedu