Familial dyslipidemia is rare compared to polygenetic causes. Nevertheless, it is important not to miss this diagnosis, as it is more strongly associated with an increased risk of early cardiovascular disease and scores for calculating cardiovascular risk are not valid in this population. Early detection and management based on lifestyle optimization and treatment of cardiovascular risk factors can delay the onset of cardiovascular complications and thus improve patients' quality of life. A LDL-Cholesterol of 4,9 mmol/l has recently been suggested as the cut-off for starting lipid lowering therapy, but remains controversial because the majority of people above this threshold do not have primary monogenic dyslipidemia. The age at which therapy should be initiated as well as the targets for treatment are also controversial.