It is widely accepted that people with Down syndrome are more likely than the general population to develop Alzheimer's dementia as they age. However, the diagnosis can be problematic in this population for a number of reasons. These include: the large intra-individual variability in cognitive functioning, the different diagnostic and methodological procedures used in the field and the difficulty in obtaining baseline levels of cognitive functioning in this population with which to assess cognitive and behavioral change. Recent researchers have begun to suggest ways around these difficulties. This review explores these recent developments and provides recommendations which may aid clinicians in their attempts to diagnose Alzheimer's dementia in the early stages in the Down syndrome population.