Fetal Alcohol Spectrum Disorder (FASD) is a preventable, lifelong disability that disproportionately affects Aboriginal and Torres Strait Islander people. This review provides a comprehensive synthesis of the available information on FASD among Aboriginal and Torres Strait Islander people, with reference to the limitations on population-based data and evaluated programs. The review outlines; the harms of alcohol use in the context of colonisation, cultural perspectives on assessment and diagnosis, effective prevention programs and a summary of state and national policies. Health impacts, educational outcomes and the effects of FASD on vulnerable populations such as children in protection and young people in the justice system, are also discussed. Specific perspectives on why women drink during pregnancy and the role of Aboriginal women in preventing FASD are explored. Recommendations include that there be greater focus on the benefits of involving men in the prevention and management of FASD and that future FASD prevention strategies for Aboriginal and Torres Strait Islander people should be community identified, led, and driven. FASD assessment, treatment and public health programs must also consider the history of trauma incurred by Aboriginal and Torres Strait Islander communities as a result of colonisation.