Lipoprotein(a) is an LDL-like molecule that contains a specific glicoprotein moiety apolipoprotein(a). Apo(a) is a very polymorphic glicoprotein and the interindividual differences in the molecular mass of its isoforms, which are themselves a consequence of genetic variability of the LPA locus, are the cause of high variability of Lp(a) serum concentration. The serum concentration of Lp(a) is, therefore, mainly (>90%) genetically determined. The causal association between high Lp(a) serum concentration and atherosclerotic cardiovascular disease and aortic valve stenosis is well established with abundant genetic evidence. Although it was once considered so, today we know that Lp(a) is not a risk factor for venous thromboembolism. According to current definitions, around 20% of the general population has a high Lp(a) concentration and for those people that is a highly significant risk factor. Measurement of serum Lp(a) concentration is recommended to be performed at least once in a lifetime, preferably alongside the first lipid profile. Considering that Lp(a) concentration is quite stable during the course of adult life, in the absence of specific Lp(a)-lowering therapy repeat measurements are not necessary. In individuals with high Lp(a) concentration it is recommmended that Lp(a) be incorporated into overall risk estimation and that other risk factors be managed accordingly. Although currently there are no approved specific Lp(a)-lowering therapies, several drugs are now being developed with very promising results. Of those, phase 3 trials are ongoing for two new drugs and they are studying their effect on cardiovascular risk in individuals with high Lp(a) concentrations.