Young children with sickle cell disease are at risk of brain damage, including stroke. We tested the hypothesis that such patients are also at risk of cognitive impairment. We characterized the cognitive ability of kindergarten children to minimize the effect of disease-related school absence. The Memphis City Schools use the Developing Skills Checklist, a teacher-administered test given in the classroom, to assess kindergarten-appropriate skills. Data were obtained for 34 patients, who were matched to controls by gender, race, date of birth, school, and approximate income. Two controls were selected for each patient, and paired t-tests were used to compare patient's scores to composite control scores. Patients scored lower than controls in auditory discrimination (P < .01), and there was a trend (P < .10) toward lower patient scores in language. Deficits cannot be attributed to school absence and may predict academic problems for patients with sickle cell disease.