The volume of the input region of the basal ganglia, the striatum, is reduced with aging and in a number of conditions associated with cognitive impairment. The aim of the current study was to investigate the relation between the volume of striatum and general cognitive ability in a sample of 303 healthy children that were sampled to be representative of the population of the United States. Correlations between the WASI-IQ and the left striatum, composed of the caudate nucleus and putamen, were significant. When these data were analyzed separately for male and female children, positive correlations were significant for the left striatum in male children only. This brain structure-behavior relation further promotes the increasingly accepted view that the striatum is intimately involved in higher order cognitive functions. Our results also suggest that the importance of these brain regions in cognitive ability might differ for male and female children.