Positron emission tomography (PET) imaging with F18-fluorodeoxyglucose (FDG) is increasingly used as an adjunct to clinical evaluation in the diagnosis of dementia. Considering that most FDG-PET studies in dementia use clinical diagnosis as gold standard and that clinical diagnosis is approximately 80% sensitive or accurate, we aim to review the evidence-based data on the diagnostic accuracy of brain FDG-PET in dementia when cerebral autopsy is used as gold standard. We searched the PubMed and Medline databases for dementia-related articles that correlate histopathological diagnosis at autopsy with FDG-PET imaging and found 47 articles among which there were only 5 studies of 20 patients or more. We were able to conclude that sensitivity and specificity of FDG-PET for Alzheimer's disease are good, but more studies using histopathological diagnosis at autopsy as gold standard are needed in order to evaluate what FDG-PET truly adds to premortem diagnostic accuracy in dementia.