Hypercholesterolemia is a major concern in the USA, with studies identifying children as young as 2 years old with early-stage atherosclerosis. Genetics play a major role in the dyslipidemia of children, but other factors, such as diet and lack of physical activity, confound the problem. Familial hypercholesterolemia (FH) is a genetic condition that causes lifelong elevations in low-density lipoprotein cholesterol (LDL-C). The heterozygous form of the disease affects around 1 in 200 people, and the homozygous form of the disease affects around 1 in 160,000–300,000 people. Early identification and appropriate management of patients with FH are essential to reduce cardiovascular disease morbidity and mortality. Consequently, US dyslipidemia guidelines recommend routine screening of all children aged 9–11 years, and that LDL-C levels should be <110 mg/dL in children and adolescents. The primary management strategy in all children with dyslipidemia is diet and lifestyle; a healthy diet (including fruits, vegetables, fish, and whole grains) and increased physical activity should be encouraged. Most patients with FH will also require pharmacotherapy to reduce LDL-C levels to ≤130 mg/dL. Statins are recommended as first-line therapy due to their proven efficacy in reducing LDL-C and improving other lipid parameters in children. They have also been shown to have a positive effect on atherosclerosis. Safety is of particular concern with children; however, studies have so far shown that the side-effect profile of statins in children is similar to that in adults. Despite improvements in disease management, FH remains underdiagnosed and undertreated, highlighting the need for greater awareness and understanding.