BACKGROUND: High Lp(a) concentrations are linked to an increased risk of cardiovascular disease (CVD). However, more evidence is needed to assess the association of Lp(a) with CVD in different vascular beds. HYPOTHESIS: The aim was to assess the prevalence of increased Lp(a) levels and the association between Lp(a) levels and CVD in hypercholesterolemic patients. METHODS: We examined 220 patients (110 women) with suspicion of FH. The mean (SD) age was 49.1 (15.02) years, LDL-C 3.49 (1.75) mmol/L, and the median (IR) Lp(a) concentration 0.15 (0.53) g/L. RESULTS: CVD was present in 24.5%, coronary artery disease (CAD) in 21.4% of the examined individuals. Patients with CVD and patients with CAD had higher Lp(a) levels than patients without these diseases (p = 0.0403 and p = 0.0063, respectively); however, after adjustment for age and sex, only the difference in Lp(a) concentrations between persons with and without CAD remained significant. In total, 42.3% of patients who underwent carotid ultrasound examination had carotid plaques. We did not observe differences in Lp(a) levels between patients with or without plaques or correlations between Lp(a) and carotid IMT. In total, 28.3% of patients had Lp(a) concentrations in the high (> 0.5 g/dL), and 9.9% in the moderate-risk category (0.3-0.5 g/L). We observed an association between Lp(a) risk categories and the presence of CVD (p = 0.003) and CAD (p = 0.0004) but not with the presence of carotid plaques. CONCLUSIONS: We found a high prevalence of increased Lp(a) levels in a group of hyperlipidemic persons and strong associations of Lp(a) risk categories with CAD but not with carotid atherosclerosis.