Background: Heart transplantation in children has revolutionized the care of young patients with congenital and acquired heart disease, with considerable improvements in survival and quality of life. Heart transplantation is now being offered as a potentially life-saving option to pediatric patients with many cardiac conditions, and yet the major limiting factor remains the lack of suitable donor hearts.Requirement: Transplant teams and UNOS (the United Network for Organ Sharing) must attempt the formidable task of balancing the needs of an individual patient with a responsibility to determine the best use of a donor heart and promote equitable donor allocation. These issues become even more salient when the transplant candidate is a child with intellectual disability (ID) or neurodevelopmental delay (NDD). In this paper, we review the literature on the clinical practice and ethical considerations surrounding heart transplantation in children, with a focus on those with ID or NDD. We also review our experience at a major center for pediatric heart transplantation.