Among critically ill patients, the risk of developing disseminated intravascular coagulation (DIC) is probably highest in neonates. Low plasma reserves in pro- and anticoagulant coagulation factors, intravascular volume contraction after birth, and a high incidence of hypoxia and sepsis in critically ill newborns rapidly lead to a decompensation of the coagulation system in this population. Global coagulation tests and single-factor plasma levels have to be interpreted in the context of age-corrected normal ranges. Platelet consumption and reduced protein C plasma levels have diagnostic value; the latter also has prognostic potential in neonates with DIC and sepsis. Therapeutic success relies heavily on reversal of the underlying condition. Some coagulation-specific therapies have been explored in small studies and case series with varying success and sometimes conflicting results. Therefore, larger controlled trials in this common and serious condition are urgently needed.