The aim of this study was to characterize a group of patients (n=8) with sickle cell disease (SCD) and ischemic stroke concerning the clinical, neurological, imaging and progressive aspects. Data were collected from records and completed with an interview of patients and their parents. In this study there were 8 patients with ages ranging from 10 to 23 years old; SCD diagnosis was given between one and two years of age with clinical features of fatigue and anemia. The stroke was ischemic in all individuals and the first cerebrovascular event occurred before 6 years of age; 3 patients had recurrence of stroke despite prophylactic blood transfusion therapy and both cerebral hemispheres were affected in 4 patients. Clinical and neurological current features observed were: acute pain crises, sialorrhea, mouth breathing, motor, and neuropsychological impairments resulting from cortical-subcortical structure lesions.6613033Earley, C.J., Kittner, S.J., Feeser, B.R., Stroke in children and sickle-cell disease: Baltimore-Washington Cooperative Young Stroke Study (1998) Neurology, 51, pp. 169-176Ohene-Frempong, K., Weiner, S.J., Sleeper, L.A., Cerebrovascular accidents in sickle cell disease: Rates and risk factors (1998) Blood, 91, pp. 288-294Balkaram, B., Char, G., Morris, J.S., Thomas, P.W., Serjeant, B.E., Serjeant, G.R., Stroke in a cohort of patients with homozygous sickle cell disease (1992) J Pediatr, 120, p. 360Ohene-Frempong, K., Stroke in sickle cell disease: Demographic, clinical and therapeutic considerations (1991) Semen Hematol, 28, p. 213Powars, D., Wilson, B., Imbus, C., Pegelow, C.H., Allen, J., The natural history of stroke in sickle cell disease (1978) Am J Med, 65, pp. 461-471Arita, F.N., (1998) Acidente vascular cerebral em crianças com doença falciforme, , Tese de Doutorado Faculdade de Ciências Médicas da Santa Casa de São Paulo. São PauloSteen, R.G., Xiong, X., Langston, M.D., Helton, K.J., Brain injury in children with sickle cell disease: Prevalence and etiology (2003) Ann Neurol, 54, pp. 564-572Harriman, L.M.F., Griffith, E.R., Hurtig, A.L., Keehn, M.T., Functional outcomes of children with sickle-cell disease affected by stroke (1991) Arch Phys Med Rehabil, 72, pp. 498-502Wang, W.C., Grover, R., Gallagher, D., Espeland, M., Fandal, A., Developmental screening in young children with sickle cell disease: Results of a cooperative study (1993) Am J Pediatr Hematol Oncol, 15, pp. 87-91Thompson Jr, R.J., Gustafson, K.E., Bonner, M.J., Ware, R.E., Neurocognitive development of young children with sickle cell disease through three years of age (2002) J Pediatr Psychol, 27, pp. 235-244Armstrong, F.D., Thompson Jr, R.J., Wang, W., Cognitive functioning and brain magnetic resonance imaging in children with sickle cell disease (1996) Pediatrics, 97, pp. 864-870Adams, R.J., McKie, V.C., Hsu, L., Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography (1998) N Engl J Med, 339, pp. 5-11Wang, W.C., Kavaar, E.H., Tonkin, I.C., High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease (1991) J Pediatr, 96, pp. 205-208Dobson, S.R., Holden, K.R., Nietert, P.J., Moyamoya syndrome in childhood sickle cell disease: A predictive factor for recurrent cerebrovascular events (2002) Blood, 99, pp. 3144-3150