Turner Syndrome (TS) is a genetic disorder affecting primarily females. It arises from a loss of X-chromosome material, most usually one of the two X chromosomes. Affected individuals have a number of distinguishing somatic features, including short stature and ovarian dysgenesis. Individuals with TS show a distinct neurocognitive profile involving visuospatial deficits and selective memory and attention difficulties. They also have a number of psychosocial and behavioral problems. They are at high risk of learning disabilities and often have a need for special education. Recent research has identified some of the genetic and hormonal mechanisms contributing to their neurobehavioral profile. This article reviews the current findings on TS and shows how understanding the impact of these factors on early brain development and later brain function might advance our understanding of learning disabilities more generally. The implications of current research findings for the daily management of children with TS in the classroom are also discussed.