The catastrophic antiphospholipid syndrome is a potentially life-threatening condition with a high mortality rate, the diagnosis of which requires a high degree of clinical awareness on the part of attending physicians. Patients with this syndrome have various symptoms in common: clinical evidence of multiple organ involvement developed over a very short time period, histopathological evidence of multiple small-vessel occlusions and laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titers. The combination of high doses of intravenous heparin, steroids, gamma-globulins and/or repeated plasma exchanges are the basic treatment of choice for all patients with this severe condition.