We have identified a 37-year-old patient suffering from two myocardial infarctions, with a markedly elevated lipoprotein(a) [Lp(a)] level and normal levels of cholesterol and triglycerides. Clinically this patient presented with xanthelasma and arcus lipoides corneae. After treatment with a cholesterol synthesis inhibitor (Pravastatin), the Lp(a) concentration in this patient was reduced significantly. This case report supports the hypothesis that elevated Lp(a) is an independent risk factor for coronary artery disease. The effect of the cholesterol synthesis inhibitor on Lp(a) may be due to the upregulation of the LDL-receptor, suggesting a role for LDL receptor in Lp(a) catabolism.