Hughes (antiphospholipid) syndrome is a condition in continuous evolution. Since it was first described almost 20 years ago, significant advances in its diagnosis and management have been made. Recently, classification criteria for definite antiphospholipid syndrome have been proposed and validated. However, there is still controversy regarding issues such as the utility of new diagnostic tests (detecting antibodies to beta(2)-glycoprotein I, phospholipids other than cardiolipin or phospholipid mixtures), the role of antiphospholipid antibodies in neurological conditions such as demyelinating disease, cognitive impairment or migraine and the optimal management of thrombosis and obstetric manifestations. These are all briefly discussed in this review.