Families of children with congenital heart disease (CHD) cope differently depending on individual and familial factors beyond the severity of the child’s condition. Recent research has shifted from an emphasis on the psychopathology of family functioning to a focus on the resilience of families in coping with the challenges presented by a young child’s condition. The increasing number of studies on the relationship between psychological adaptation, parental coping and parenting practices and quality of life in families of children with CHD necessitates an in-depth re-exploration. The present study reviews published literature in this area over the past 25 years to generate evidence to inform clinical practice, particularly to better target parent and family interventions designed to enhance family coping. Twenty-five studies were selected for inclusion, using the PRISMA guidelines. Thematic analysis identified a number of themes including psychological distress and well-being, gender differences in parental coping, and variable parenting practices and a number of subthemes. There is general agreement in the literature that families who have fewer psychosocial resources and lower levels of support may be at risk of higher psychological distress and lower well-being over time, for both parent and the child. Moreover, familial factors such as cohesiveness and adaptive parental coping strategies are necessary for successful parental adaptation to CHD in their child. The experiences, needs and ways of coping in families of children with CHD are diverse and multi-faceted. A holistic approach to early psychosocial intervention should target improved adaptive coping and enhanced productive parenting practices in this population. This should lay a strong foundation for these families to successfully cope with future uncertainties and challenges at various phases in the trajectory of the child’s condition.