Sickle cell disease is one of the main causes of ischemic stroke in the childhood, generally related with stenosis of the arteries of the anterior carotid circulation. We present the results of the studies carried out in a patient with sickle cell with no history of stroke, in treatment with hidroxyurea. Transcraneal Doppler and Magnetic Resonance Imaging demonstrated the existence of stenosis in medial cerebral arteries and a Moyamoya pattern in the cerebral circulation with hemodynamic ischemic lesions.