Some children have genetically determined dyslipidemias that predisposes them to early heart attacks. In addition, dyslipidemias associated with obesity and diabetes mellitus are being increasingly recognized in children and adolescents. Postponing treatment of severely elevated cholesterol until middle-age or late adulthood in both situations may be too late for some children. It is now recommended that all children should be screened for lipids between ages 9 and 11 years and once again between ages 17 and 21 years. Selective screening in other age groups may be desirable if childhood hypertension, diabetes, or obesity is present. Evaluation for a cause is necessary in children with abnormal lipid levels. Severe elevation is likely to be genetic in origin. The mainstay of treatment of high cholesterol levels in children and adolescents is diet and physical activity. No dietary restriction is recommended below 2 years of age. When cholesterol levels are very high and presumed or known to be genetic, children above 8–10 years of age may benefit from statin therapy; however, the dosing should be conservative. Routine health monitoring should include assessment of growth and development and annual liver functions. Bile acid sequestrants and ezetimibe are also approved for children. For homozygous familial hypercholesterolemia (HoFH), the only therapy available is lipoprotein apheresis (LA). LA is presently not available in India. Recently, US Federal Drug Agency has approved mipomersen and lomitapide aimed at reducing cholesterol in HoFH.