Understanding illness perception is crucial to ensure healthy lifestyles. However, it has been poorly explained to inform effective lifestyle modification of patients with Familial Hypercholesterolaemia (FH) Therefore, we described the illness perception of different clinical categories of FH patients (using the Dutch Lipid Clinic Criteria (DLCC) and investigate its association with sociodemographic and illness-related factors. Illness perception of FH patients varies according to the level of education and the presence of CVD. Patients with ‘definite’ FH have the highest emotional disturbances. These findings may help clinicians to improve the interventions suitable for FH patients.