Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by a variety of clinical manifestations. Recent studies suggest arterial stenosis is involved in APS. Furthermore, arterial stenosis may be a relevant and treatable cause for hypertension, renal, CNS and gastrointestinal manifestations of APS. Our objective was to overview the published data regarding arterial stenosis in APS--the clinical presentation, diagnosis, suggested therapies and the possible mechanisms. We searched PUBMED (1951-2006) reference lists for the role of arterial stenosis in APS. APS patients might present with arterial stenosis. Its recognition has important diagnostic and therapeutic implications. Articles suggest that anticoagulation treatment with INR above three may reverse the artery stenosis and achieve subsequent clinical improvement. Currently, there are no specific guidelines for the management of APS patients with arterial stenosis. The exact mechanism of arterial stenosis in APS patients remains unclear.