Lipoprotein(a) is a cholesterol-enriched lipoprotein, consisting of a covalent linkage joining the unique and highly polymorphic apolipoprotein(a) to apolipoprotein B100, the main protein moiety of low-density lipoproteins. Although the concentration of lipoprotein(a) in humans is mostly genetically determined, acquired disorders might influence synthesis and catabolism of the particle. Raised concentration of lipoprotein(a) has been acknowledged as a leading inherited risk factor for both premature and advanced atherosclerosis at different vascular sites. The strong structural homologies with plasminogen and low-density lipoproteins suggest that lipoprotein(a) might represent the ideal bridge between the fields of atherosclerosis and thrombosis in the pathogenesis of vascular occlusive disorders. Unfortunately, the exact mechanisms by which lipoprotein(a) promotes, accelerates, and complicates atherosclerosis are only partially understood. In some clinical settings, such as in patients at exceptionally low risk for cardiovascular disease, the potential regenerative and antineoplastic properties of lipoprotein(a) might paradoxically counterbalance its athero-thrombogenicity, as attested by the compatibility between raised plasma lipoprotein(a) levels and longevity.