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It results in marked elevation in plasma low\u2010density lipoprotein cholesterol levels from birth that predisposes individuals to premature coronary heart disease in adult life. The majority of children and adolescents with <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> are undiagnosed, as symptoms and signs only develop after decades of hypercholesterolaemia. Cascade screening of family members after detecting <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> in an index case is an effective approach that allows the diagnosis of <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> to be made in the young, before significant atherosclerosis develops. With the availability of effective therapies, mainly statins, paediatricians are ideally placed to improve the outcomes of this disorder by detecting and managing hypercholesterolaemia in childhood, thereby preventing premature coronary artery disease. 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