Current concepts of etiology and pathophysiology resulting in disseminated intravascular coagulation (DIC) form the basis of treatment of this hemostatic disorder. Due to the heterogeneous triggering diseases and different kinds of DIC, clinical symptoms such as predominant bleeding, thromboembolic complications or organ failure, clinical experience together with the profile of laboratory test results and their development over time provide the basis for the individually tailored treatment strategy. The guiding principle of therapy is to identify and vigorously treat the underlying cause of DIC without delay. Treatment options to correct the hemostatic defect and to dampen the intravascular clotting/fibrinolytic process include transfusion of blood products, heparin, antithrombin III, and antifibrinolytic agents. The availability of new drugs such as activated protein C, tissue factor pathway inhibitor, hirudin, or synthetic serine protease inhibitors, and the upcoming trials investigating the role of these and older treatment options will help us to more clearly recommend therapy in DIC of different etiology.