Factor V (FV) deficiency (F5D) is a rare hematological disorder with a variable spectrum of bleeding manifestations. Until now, no specific protocols for the management of these patients have been established. However, available literature suggests that perioperative infusion of fresh frozen plasma (FFP) may help maintain FV levels to prevent bleeding. We present the case of a 64-year-old man with previously undiagnosed severe FV deficiency and mild FV inhibitor, who underwent aortic valve replacement with no bleeding complications.