Background: Individuals with Down syndrome (DS) are at high risk of dementia which is difficult to diagnose in DS. Neuroimaging has been identified as a potential tool to aid diagnosis by detecting changes in brain function. We carried out a review comparing functional neuroimaging in DS individuals with and without dementia. Summary: A literature search was conducted using PubMed to identify relevant studies. In DS subjects with dementia, fluorodeoxyglucose-positron emission tomography (PET) studies showed glucose hypometabolism particularly in the parietal and/or temporal regions whilst magnetic resonance spectroscopy studies showed increased myoinositol and decreased N-acetylaspartate. Ligand-based PET studies revealed significant Pittsburgh compound B binding in DS subjects over the age of 40, particularly if they had dementia. Key Messages: Neuroimaging may aid the early detection of dementia in DS; however, further longitudinal studies are required.