The coagulation cascade involves sequential enzymatic activations of serine protease zymogens that converge on the generation of thrombin. Factor V (FV) takes part in this process as a component of the prothrombinase complex. Besides its role as procoagulant factor, it is also involved in the physiologic anticoagulant pathway, by participating in the inactivation of activated factor VIII (FVIIIa). Given the dual role of FV, genetic defects in FV gene may result in opposite hemorrhagic or thrombotic phenotypes. This review focuses on the structure, function (procoagulant and anticoagulant), regulation (activation and inactivation) of FV as well as on the genetic defects associated with mutations in the FV gene.