Long-term transfusion therapy in patients with sickle cell disease and stroke markedly decreases the risk of stroke recurrence. However, it is not known how long the transfusions should be continued. Published reports have documented a high risk of stroke recurrence after stopping transfusion. We report on nine consecutive patients with sickle cell disease and stroke whose long-term transfusion therapy was discontinued and in whom no ischemic strokes developed during 80.75 patient years of follow-up.