We report the case of a 52-year-old male patient with recurrent thrombosis from ‘primary antiphospholipid syndrome’ who developed renal microangiopathy. Despite anticoagulant therapy with coumadin, serum creatinine progressively increased from 398 to 592 μmol/l and platelets decreased to 43,000. The patient responded to high-dose methylprednisolone and aspirin and the renal function improved. A review of the literature disclosed 4 other cases of association between primary antiphospholipid syndrome and renal microangiopathy. The clinical characteristics of these cases are discu