Background <p>Early identification and treatment of lipid disorders has the potential to improve long-term cardiovascular health in affected individuals. We performed a systematic review of guidelines on screening, diagnosis, and management of dyslipidaemias in children.</p> Methods <p>We searched Medline, Embase, and selected websites for relevant clinical practice recommendations/guidelines, published in English/German from 01.01.2018 to 31.01.2025. We used AGREE II tool to assess guidelines’ quality.</p> Results <p>Six national guidelines, mainly on familial hypercholesterolemia (FH), were included. Recommendations for universal screening for dyslipidaemias in childhood differed, ranging from the statement of insufficient evidence (<i>n</i> = 1) or negative recommendation of the national screening committee (<i>n</i> = 1) through weak (<i>n</i> = 2) to moderate (<i>n</i> = 1) recommendations to consider population screening. All organizations recommended statins as a first-line drug therapy in children with FH (strong recommendation). Behavioral intervention was mainly strongly recommended as an additional treatment component of FH, and as therapy of choice in childhood obesity related dyslipidaemia. Guidelines applied different systems to categorize certainty of evidence and strength of recommendations. 50% of guidelines scored high (&gt;70%) for rigor of development.</p> Conclusions <p>Current guidelines provide overall consistent recommendations for the management of childhood dyslipidaemias, but show lack of consensus regarding universal screening for early detection of lipid disorders.</p> Impact <p><UnorderedList Mark="Bullet"> <ItemContent> <p>Health behavior counseling is recommended for all children affected with dyslipidaemia.</p> </ItemContent> <ItemContent> <p>Statins are consistently proposed as a first-line drug therapy in pediatric familial hypercholesterolemia.</p> </ItemContent> <ItemContent> <p>Recommendations for universal screening for dyslipidaemias in childhood vary.</p> </ItemContent> <ItemContent> <p>This systematic review provides insights into current recommendations on pediatric lipid disorders, explores potential reasons for discrepancies, and indicates the need for consensus on the optimal strategy for early identification of familial hypercholesterolemia.</p> </ItemContent> </UnorderedList></p>