Drs. R. Michael Scott and Edward R. Smith discuss the surgical treatment of moyamoya syndrome in patients with sickle cell anemia. Successful care in these complicated cases involves multidisciplinary cooperation among hematologists, anesthesiologists, and neurosurgeons. The authors recommend operative treatment with pial synangiosis, which appears to be safe and to confer long-lasting protection against further stroke in this population. The particulars of operative management and postoperative care are discussed.