Familial hypercholesterolemia (FH) is one of the most common genetically determined diseases. Its most serious complications are premature coronary artery disease and myocardial infarction. Myocardial infarction and sudden cardiac death are the most common causes of death among patients with FH. It often afflicts young patients. Concurrently diagnosis of FH families and an early implementation of appropriate treatment offer the opportunity to significantly delay or prevent development of full-blown atherosclerosis. The availability of effective treatments should be an incentive to search for this group of young people burdened with very high cardiovascular risk before they experience symptoms of the disease.