The catastrophic antiphospholipid syndrome (CAPS) is a life-threatening sub-entity of the antiphospholipid syndrome (APS) resulting in multiorgan failure and a mortality rate that may reach 50%. The optimal treatment regimen for CAPS is unknown. To evaluate the benefit of therapeutic plasma exchange (TPE) therapy in CAPS, we undertook a computer-assisted (MEDLINE, National Library of Medicine, Bethesda, MD) search of the literature to locate all cases of CAPS (case reports and reviews) treated with TPE. In view of the published literature so far, TPE has shown reasonable therapeutic benefits and improved survival for patients with CAPS. The use of this technique, along with anticoagulation and if needed pharmacological immunosuppression, should be considered for the acute management of patients who present with this life-threatening condition.