Aim: to analyze adherence of FH patients with familial hypercholesterolemia (FH) to the statin therapy and reveal factors, which influence it; to assess the degree of target level of low-density lipoprotein cholesterol (LDLCH) achievement by FH patients on statin therapy. Materials and methods. We included in this study 203 FH patients aged >18 years (mean age 50.0±1.1 years, 82 men). Definite FH was diagnosed in 96 persons, in the other patients FH was considered possible. For evaluating the adherence to therapy with statins we used the Morisky-Green questionnaire. Results. Among patients with definite FH 57 % were adherent to lipid-lowering therapy, 16 % were partially adherent, and 27 % – not adherent. Target LDLCH levels were achieved in 22.6 % and 12.5 % of patients with definite and possible FH, respectively. Smoking and gender were not associated with adherence to statin therapy. Factors associated with higher adherence were age (p=0.000003), arterial hypertension (odds ratio [OR] 1.90, 95 % confidence interval [CI] 1.02 to 3.55], p=0.044), ischemic heart disease (IHD) (OR=2.99, 95 %CI 1.50 to 5.97, p=0.002), history of myocardial infarction (MI) (OR 5.26, 95 %CI 2.03 to 13.60, p=0.0006), history of myocardial revascularization (OR 20.3, 95 %CI 2.64 to 156.11, p=0.004) and the fact of achieving target LDLCH level (OR 19.93, 95 %CI 7.03 to 56.50, p<0.0001). The main reason for the refuse from statin therapy in 87 % of patients was fear of side effects. Main reasons for stopping of ongoing therapy were: myalgia, an increase in transaminases, skin rashes, and high cost in 12, 35, 12, and 6 % of patients, respectively. The decision to withdraw therapy with statins was made by 29 % of patients by themselves. Conclusion. In this study 57 % of patients with definite FH were adherent to statin therapy. Factors associated with increased adherence were age, hypertension, IHD, history of MI, history of myocardial revascularization, achievement of target LDLCH level. Target LDLCH levels were achieved by 22.6 and 12.5 %% of patients with definite and possible FH, respectively.