Background: Fetal Alcohol Spectrum Disorders (FASD) affect a significant number of children in this country. This article addresses diagnostic issues related to fetal alcohol syndrome (FAS) and other alcohol-related disabilities, discusses associated features and behaviors of FASD, and introduces interventions to support children with FASD in school settings. Methods: A comprehensive review of FAS and FASD literature as it relates to school functioning was conducted. Results: Prenatal alcohol exposure can result in a broad range of negative developmental consequences, including deficits in cognitive and academic functioning, psychological disorders, behavioral problems, and difficulties with independent living. Children with prenatal alcohol exposure are at risk for a spectrum of difficulties at school. Conclusions: This topic is of considerable relevance to all professionals in a school setting, including teachers, administrators, school psychologists, special education providers, special service providers, and school nurses who interact with children who may be prenatally exposed to alcohol. Successful interventions will need to balance the use of environmental modifications, immediate and meaningful positive and negative consequences for behaviors, and opportunities to teach children skills to monitor and modify their behavior.