Familial dyslipidemia, a frequent genetic cause of premature cardiovascular disease, is still underdiagnosed and undertreated. According to recent European studies, the prevalence of familial hypercholesterolemia is higher than previously suspected and reaches 1/200-300 persons, while familial combined hyperlipidemia affects 1-3% of the population. Screening is important, as familial dyslipidemia often leads to cardiovascular event before 60 years of age, and usual scores of risk are not appropriate for these patients. Screening is recommended in childhood in case of family history of premature cardiovascular disease or severe hyperlipidemia in first degree relatives. Lifestyle modifications, eviction of any additive cardiovascular risk factor and tailored drug therapy are the cornerstones of an optimal management.