William's Syndrome (WS) is a rare neurodevelopmental disorder caused by a genetic abnormality, affecting about 1 in 10,000 people worldwide. While there are some behavioral similarities between WS and other high incidence disabilities, such as autism, several unique physical, cognitive, and behavioral characteristics are expressed in individuals with WS. Many of the cognitive and behavioral characteristics of individuals with WS are directly associated with neuroanatomical abnormalities. In order to best support students with WS in school settings, school psychologists must be able to recognize the distinctive characteristics of students with WS and utilize their specific areas of strength. Implications and suggestions for assessment and interventions practices are discussed.