Familial hypercholesterolemia (FH)is a genetic disorder that causes elevated levels of low-density lipoprotein cholesterol (LDL-C), increasing the risk of premature cardiovascular disease (CVD) at an early age. FH is classified into two forms: heterozygous FH (HeFH) and homozygous FH (HoFH). The identification of FH in children is crucial, as untreated patients have a higher risk of developing CVD than the general population. Statins are the first-line agents for FH treatment in children and aim to lower LDL-C levels and reduce the risk of CVD, especially in young adults. This review aims to provide a rationale for statin therapy in the management of pediatric FH and discusses the potential benefits and risks associated with statin efficacy. The article also discusses the overview of statins, statin potency, and the potential risks associated with statin therapy.