Lipoprotein(a) (Lp[a]) is a risk factor of cardiovascular disease (CVD). Familial hypercholesterolemia (FH), which exhibits high low-density lipoprotein cholesterol (LDL-C) levels, is a risk factor of CVD. The relationship of Lp(a) with CVD has been characterized in populations specific to FH. Studies reporting on the relationship of Lp(a) with CVD among FH subjects via PubMed up to 2020 were reviewed. Eight studies were identified as eligible. In the meta-analyses, a high Lp(a) level was significantly and predictively associated with CVD compared to a low Lp(a) level in 2 cross-sectional studies (odds ratio = 2.57; 95% confidence interval (CI): 1.16-5.73) and 6 cohort studies (risk/hazard ratio = 1.91; 95% CI: 1.50-2.43). The totally integrated relative risk of these studies was 1.97 (95% CI: 1.57-2.46). FH subjects with high Lp(a) levels can have a high CVD risk, and besides LDL-C, attention should be paid to Lp(a) levels in FH subjects. Copyright: © 2020 Termedia & Banach.