Disseminated intravascular coagulation (DIC) is a condition that involves concurrent (microvascular) thrombosis and widespread bleeding complications. The prothrombotic tendency in DIC arises from a systemic coagulation activation, whereas continuing the use of platelets and clotting factors is responsible for a consumption coagulopathy that enhances the risk of hemorrhage, the latter in particular in hyperfibrinolytic types of DIC. DIC does not occur by itself but is always associated with another condition, such as severe infection, malignant disease, serious (poly)trauma, or obstetric complications. In recent years, a much better understanding of underlying pathways leading to the coagulopathy of DIC has been identified, including tissue factor-dependent initiation of coagulation, enhanced platelet-vessel wall interaction, loss of natural anticoagulant function, and the bidirectional interplay between inflammation and coagulation. In practice, a reliable diagnosis of DIC can be established with simple scoring algorithms that utilize readily available laboratory tests, which are likely to be routinely available in virtually all hospitals. The basis of the management of DIC is the explicit and thorough treatment of the underlying disorder. Adjunctive strategies focused at the impairment of coagulation, including transfusion practices, anticoagulants, and restoration of physiological anticoagulant mechanisms, may be indicated in specific situations but have not been definitively established based on convincing results from clinical trials.