Antiphospholipid syndrome is often recognize autoimmune disease associated with arterial and venous thrombosis and pregnancy loss. It is caused by antibodies against proteins combined with cell membrane anionic phospholipids. Kidney are one of the most often affected organs in course of antiphospholipid syndrome. Renal manifestations include thrombotic microangiopathy and large vessel thrombosis. In patients with end-stage renal disease, antiphospholipid antibodies are prevalent and may increase in frequency with time on dialysis. The presence of anticardiolipin antibodies have been associated with high incidence of hemodialysis access clotting. In renal transplant recipients, the incidence of antiphospholipid antibodies is also elevated and may be associated with higher incidence of primary graft non-function. Treatment of antiphospholipid syndrome remains centered around anticoagulation. The use of immunosuppressive agents could be very useful. Early detection of renal involvement and treatment may improve the prognosis of these patients.