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Introduction: In phenylketonuria (PKU) changes in dietary patterns and behaviors in sapropterin-responsive populations have not been widely reported. We aimed to assess changes in food quality, mental health and burden of care in a paediatric PKU sapropterin-responsive cohort. Methods: In an observational, longitudinal study, patient questionnaires on food frequency, neophobia, anxiety and depression, impact on family and burden of care were applied at baseline, 3 and 6-months post successful sapropterin-responsiveness testing (defined as a 30% reduction in blood phenylalanine levels). Results: 17 children (10.8 ± 4.2 years) completed 6-months follow-up. Patients body mass index (BMI) z-scores remained unchanged after sapropterin initiation. Blood phenylalanine was stable. Natural protein increased (<i>p</i> < 0.001) and protein substitute intake decreased (<i>p</i> = 0.002). There were increases in regular cow’s milk (<i>p</i> = 0.001), meat/fish, eggs (<i>p</i> = 0.005), bread (<i>p</i> = 0.01) and pasta (<i>p</i> = 0.011) intakes but special low-protein foods intake decreased. Anxiety (<i>p</i> = 0.016) and depression (<i>p</i> = 0.022) decreased in caregivers. The impact-on-family, familial-social impact (<i>p</i> = 0.002) and personal strain (<i>p</i> = 0.001) lessened. After sapropterin, caregivers spent less time on PKU tasks, the majority ate meals outside the home more regularly and fewer caregivers had to deny food choices to their children. Conclusion: There were significant positive changes in food patterns, behaviors and burden of care in children with PKU and their families after 6-months on sapropterin treatment.