During the last decade, antiphospholipid antibodies and their clinical manifestations have given rise to increasing interest and have been associated with a wide spectrum of clinic expression, including arterial and venous thrombosis, thrombocytopenia, recurrent fetal wastage, Coombs positive haemolysis, livedo reticularis and neurological abnormalities, commonly present as isolated recurrent events, and rarely characterized by fatal outcome. Recently, an acutely disseminated vasculopathy, the so-called "catastrophic antiphospholipid syndrome" (CAS) characterized by non-inflammatory vascular occlusion and frequency of fatal outcome, has been described. We present yet another case report of this new and poorly understood entity and review its antecedents, clinical manifestations, serological profile, treatment and outcome.