ABSTRACT
Phenylketonuria (PKU) is an autosomal recessive disease caused by variants in the gene that encodes phenylalanine hydroxylase (PAH), limiting the metabolism of phenylalanine (Phe). When PAH activity is absent or hindered, Phe is not converted to tyrosine, leading to an accumulation of Phe in the blood, which can cause serious neurological complications. Once PKU is diagnosed, treatment should be started immediately, and the basis for this is dietary restriction of foods with high levels of Phe, associated with the use of protein substitutes and intake of foods with low protein content. This restriction accompanies patients throughout their lives, making their diets unpalatable and monotonous, which represents a major challenge for health professionals and patients, considering that these factors favor food transgression. In this context, the objective of this work was to carry out an integrative review based on evidence regarding the intake of fruits and vegetables, by phenylketonurics, taking into account the greater or lesser tolerance to Phe. Since, some researchers have dedicated themselves to evaluating the biochemical effect of unrestricted consumption of fruits and vegetables at PKU, unifying the information in this regard. It was observed that the intake of vegetable protein by patients with PKU has shown to be promising since the studies indicate that the intake of these proteins does not present adverse effects to the metabolic control of the Phe.