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Objective Children with sickle cell disease (SCD) exhibit poor somatic growth due to nutritional and metabolic effects, but potential relationships between growth and other areas of development are unclear. We examined whether growth is related to cognition and whether growth might be one marker of neurocognitive risk. Methods Sixty-four children with SCD and eighty-one demographically similar controls, ages 4 to 8 years, completed cognitive and anthropometric measures. Results Height-for-age partially accounted for cognitive decrements related to SCD on all cognitive measures. Higher body-mass-index was a significant predictor of higher visual-motor and academic achievement scores in children with SCD, but not in controls. Conclusions In some children with SCD, especially those with HbSS and Hb Sβ0, low height-for-age may help to explain neurocognitive risk. Higher body-mass-index may be related to better cognitive outcomes in children with SCD. Nutrition deficits in SCD could explain the association between somatic growth and cognitive deficits.