Since the 2016 release of the Australian Guide to the Diagnosis of Fetal Alcohol Spectrum Disorder (FASD), considerable progress has been made in the identification and diagnosis of the disorder. As part of a larger process to review and update the Guide, the aim of this study was to identify review priorities from a broad range of stakeholders involved in the assessment and diagnosis of FASD. Sixty-two stakeholders, including healthcare practitioners, researchers, other specialists, individuals with cultural expertise, lived experience and consumer representatives completed an online survey asking them to describe up to five priorities for the review of the Australian Guide to the Diagnosis of FASD. A total of 267 priorities were described. Content analysis of responses revealed priority areas relating to diagnostic criteria (n = 82, 30.7%), guideline content (n = 91, 34.1%), guideline dissemination (n = 15, 5.6%) and guideline implementation (n = 63, 23.6%). Other considerations included prevention and screening of FASD (n = 16, 6%). Engaging stakeholders in setting priorities will ensure the revised Australian Guide can be as relevant and meaningful as possible for the primary end-users and that it meets the needs of individuals with lived experience who will be most affected by the diagnosis.