Lipoprotein(a) (Lp(a)) is a low density lipoprotein-like particle in which apolipoprotein B100 is covalently linked to the unique apolipoprotein(a). There is a mounting body of evidence suggesting a role of Lp(a) in the development and progression of several vascular diseases, such as coronary heart disease, ischemic stroke, abdominal aortic aneurysm and venous thromboembolism, so that prominent scientific societies have recently endorsed guidelines and recommendations that increasingly encourage the screening and the therapeutic management of Lp(a) excess. In this article, we review the epidemiologic evidence, guidelines and recommendations concerning the relationship between increased plasma Lp(a) levels and risk of cardiovascular disease or venous thromboembolism by systematically retrieving the most relevant articles from electronic databases. Although uncertainty still remains regarding the opportunity to screen for hyperlipoproteinemia(a), it seems inopportune as yet to measure plasma Lp(a) levels in asymptomatic persons, while its measurement might be of clinical significance in selected categories of patients at intermediate or high cardiovascular risk. The measurement of Lp(a) should be performed by using immunometric, harmonized and size-insensitive techniques and results reported in total lipoprotein mass rather than in traditional units. It is uncertain if Lp(a) genotyping or phenotyping add any additional information for the cardiovascular disease risk stratification. Although the optimal therapeutic management of Lp(a) excess is still controversial, a general agreement exists that very high Lp(a) levels should be lowered in patients with multiple cardiovascular risk factors, preferably with nicotinic acid therapy (e.g., 1.0-3.0 g/day).