We review current concepts regarding the genetic, structural and metabolic features of lipoprotein(a), a major inherited cardiovascular pathogen. Although lipoprotein(a) is almost completely confined to a subset of primates, the hedgehog produces a lipoprotein(a)-like complex, which appears to have evolved independently from that of humans. The physiological role of lipoprotein(a) in humans is still unclear, and individuals with low or null concentrations of plasma lipoprotein(a) manifest no deficiency syndrome or disease. The integration of recent discoveries about the structure and metabolism of this unique lipoprotein particle has allowed the formulation of some hypotheses concerning the evolutionary advantages of synthesizing lipoprotein(a)-like particles.