Every day, thousands of children suffer the effects of chronic health conditions and families struggle with illness management and children’s behavioural and emotional adjustment. Many parents experience difficulties with their caregiving role and lack confidence in their ability to manage their child’s illness and ensure the child’s well-being. While there is consistent evidence as to the extent and impact of childhood chronic illness, there is a paucity of evidence-based parenting approaches to help children with chronic health conditions and their families. This paper provides a narrative review of the current literature to examine relationships between chronic childhood illness, emotional and behavioural disorders and parenting. Key guidelines and recommendations for the development of evidence-based parenting programs for parents of children affected by chronic health conditions are provided.