The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe and acute form of APS with multiorgan involvement. Patients with this condition have the following features in common: a) clinical evidence of multiple organ involvement developed over a very short time period; b) histopathological evidence of multiple small vessel occlusions, and c) laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titer. The two key pathobiologic mechanisms that cause the clinical manifestations of catastrophic APS are the development of thrombosis and the systemic inflammatory response syndrome. A precipitating factor is reported in more than half of patients. A combination of anticoagulants, corticosteroids, intravenous immunoglobulins and/or plasma exchanges are the treatment modalities advocated for all patients with this severe condition. Unfortunately, its mortality is still high (around 30%) despite therapy. However relapse is uncommon.