PURPOSE OF REVIEW: This review aims to summarize current advances in children and adolescent hyperlipidemia, and re-evaluate the unique epidemiology, genetic and environmental causes, and pathophysiological mechanisms of the condition. RECENT FINDINGS: Although lipid-lowering drugs have significantly reduced the burden associated with atherosclerotic cardiovascular diseases in adults, the metabolic cardiovascular risks among children and adolescents continue to increase, indicating an underestimated gap in our understanding of childhood hyperlipidemia, in particular the characteristics of the developmental pathway in the early stages of life. We summarized recent evidence that incorporates intrauterine metabolic imprinting, metabolically healthy obesity, and other intrinsic and extrinsic factors as interdependent causes of dyslipidemia in pediatric age.  Hyperlipidemia in pediatric age is not simply an early onset of adult diseases, but a distinctive entity shaped by developmental programming, dynamic hormonal changes, intestinal microbiota interaction, and the unique social environment. We call for a re-evaluation to the framework for the lipid management in children and adolescents, including the screening measures, mechanism research, and intervention strategies more centered on the developmental origin and the plasticity in the early stages of life. The limitations of the current research and possible directions are also discussed.