Patients presenting for cardiac surgery with hypercoagulable states pose a challenge. Although preoperative functional and genetic screening for hypercoagulable disorders may provide pathophysiologic information, it is difficult to make preoperative hematologic management plans in the absence of consensus guidelines, prospective studies, or case reports. We document the first case of prothrombin 20210A mutation in a patient undergoing elective aortic valve replacement. Peri-procedural bridging with heparin and avoidance of antifibrinolytic agents represent salient features of our successful perioperative management.