Genetic variants in the coagulation system have been known since antiquity. Today, because of modern improvements in diagnosis and medical management, the clinician is likely to encounter a spectrum of coagulation factor deficiencies and identified polymorphic variants in the surgical population. Because perioperative hemorrhagic and thrombotic complications are potentially serious, it is important to understand the role that these defects and variants may play in predicting risk and optimizing patient management. The implications of coagulation genetics on the perioperative management of the cardiac surgery patient are reviewed.