End stage liver disease results in a complex and variably severe failure of hemostasis that predisposes to abnormal bleeding. The diverse spectrum of hemostatic defects includes impaired synthesis of clotting factors, excessive fibrinolysis, disseminated intravascular coagulation, thrombocytopenia, and platelet dysfunction. Hemostasis screening tests are used to assess disease severity and monitor the response to therapy. Correction of hemostatic defects is required in patients who are actively bleeding or require invasive procedures. Fresh frozen plasma, cryoprecipitate, and platelet transfusion remain the mainstays of therapy until larger trials confirm the safety and efficacy of recombinant factor VIIa in this population.