Congenital heart disease (CHD) is the most common birth defect and is associated with significant morbidity and mortality. With advances in medical management and surgical techniques, most individuals with CHD now live into adulthood. Many children and adults with CHD face ongoing medical, developmental, psychological, and psychosocial challenges. Recognizing and mitigating neurodevelopmental abnormalities is an important component of the holistic care of children and adults with CHD. This article outlines the scope of neurodevelopmental abnormalities seen in children and young adults with CHD, individual risk stratification, screening and evaluation recommendations, and emerging strategies to improve neurodevelopmental outcomes. Pediatricians play a central role in identifying high-risk patients and referring for neurodevelopmental evaluation, screening low-risk patients, supporting neuroprotective practices in hospitalized patients, coordinating developmental services, and supporting children and families as they navigate the sequalae of CHD.