Abstract The low-density lipoprotein receptor-related protein 1 (LRP1) is a multifunctional cell surface receptor member of the low-density lipoprotein (LDL)-receptor family. As LRP1 plays an important role in endocytosis and regulation of signalling pathways, it is implicated in a number of physiologic processes, including the regulation of lipid metabolism, the proliferation of vascular smooth muscle cells and in neuro-development. More recently, LRP1 has been implicated in the catabolism of factor VIII and regulation of its plasma concentrations. The pathophysiology of the role of LRP1 in hemostasis will be summarized in this review.