Forty-seven patients with severe acute disseminated intravascular coagulation (DIC) were identified during a period when total hospital admissions were 115,175. Infections, shock, trauma, hepatic disease and malignancy were the common predisposing factors. Most patients were critically ill. Routine treatment included aggressive therapy of the underlying diseases and the administration of blood products, vitamin K and folic acid when indicated. Twelve patients were treated with heparin; bleeding worsened in seven (58 per cent), the DIC diminished in five (42 per cent), and 10 patients (83 per cent) died. Thirty-five patients did not receive heparin; the DIC diminished in 13 (37 per cent), and 30 patients (86 per cent) died. These deaths would probably not have been prevented by heparin therapy. Microvascular thrombi were not found in 25 patients who were examined at autopsy although infarcts possibly secondary to them were found in four. Hpwever, in three of these patients local disease was a more likely cause. Infarcts, presumably secondary to microthrombi, were diagnosed clinically in three additional patients. Acute renal failure occurred in 19 patients and acute respiratory failure in 16. Sepsis or shock was present in each of these patients; in those examined at autopsy neither microthrombi nor infarction could he implicated as the likely cause of organ failure. Eight patients had venous thromboemboli, and seven had other large vessel thrombi. All of the latter were associated with local disease. Potentially serious hemorrhage occurred in 33 patients primarily from wounds, the gastrointestinal tract and lungs. Local disease was found when adequate investigations were performed. Although bleeding (12 patients) and thrombosis (eight patients) often contributed to death, they were never the sole cause and were the immediate cause in only two patients. These findings suggest that severe acute DIC is uncommon, is not necessarily associated with microvascular thrombosis, rarely causes significant organ damage, and seldom eventuates in severe hemorrhage or large vessel thrombi unless local disease is also present. Heparin is rarely beneficial and often worsens bleeding. The high mortality associated with severe acute DIC cannot readily be attributed to it. It is perhaps best regarded as an incidental, often preterminal event, occurring in many acute catastrophic illnesses. © 1979.