Luijten J, et al. - During a follow-up in familial combined hyperlipidemia (FCHL) patients, their normolipidemic (NL) relatives and spouses, researchers assessed the incidence of cardiovascular disease (CVD) in this era of protocolled, primary prevention, as well as investigated if cardiovascular risk estimation based on the Systemic Coronary Risk Estimation (SCORE) chart is justified in FCHL. The participants were originally a part of the baseline cohort in 1998–2005 (n = 596). For 85% of the original cohort, follow-up data (median: 15 years) was analyzed. Findings revealed a substantially increased risk of primary CVD in FCHL patients, despite preventive measures. In FCHL, the SCORE chart did not lead to an overestimation of CVD risk– a nowadays frequently observed phenomenon thanks to improved primary prevention. It is justifiable to use more aggressive treatment to avoid excessive CVD in FCHL.