Lipoprotein Lp(a) excess has been identified as a powerful predictor of premature atherosclerotic vascular disease in several large, prospective studies. Lipoprotein Lp(a) levels modulate the risk of coronary heart disease in patients with hypercholesterolemia, and lipoprotein Lp(a) excess is commonly detected in men and women with premature coronary atherosclerosis. Lipoprotein Lp(a) contributes to atherothrombotic risk by multiple mechanisms that include impaired fibrinolysis, increased cholesterol deposition in the arterial wall, and enhanced oxidation of low density lipoprotein cholesterol. Although low density lipoprotein cholesterol reduction is the primary intervention in patients with lipoprotein Lp(a) excess, specific therapy to lower lipoprotein Lp(a) may be indicated for patients with premature coronary atherosclerosis, a strong family history of premature atherosclerosis, or refractory hypercholesterolemia. In consideration of the high prevalence of lipoprotein Lp(a) excess in patients with premature coronary heart disease and the intricate role of lipoprotein Lp(a) in atherothrombosis, this review provides an evidence-based approach to the screening and treatment of patients with lipoprotein Lp(a) excess.