This study examined the use of neuropsychological tests to assist in the differential diagnosis of dementia among persons with mental retardation. The author compared performances of persons with mental retardation and dementia ("n" = 10) to persons with mental retardation without dementia ("n" = 12). Participants were matched by IQ (mild or moderate mental retardation), age, presence of Down syndrome, and gender. In addition, all participants in the dementia group had corroborative medical tests (i.e., imaging, EEG, or high tau low AB42 protein testing) consistent with diagnosis of dementia. Test performance was compared on measures of attention and executive functions, language, memory and learning, and a dementia screening. Results from MANOVAs and nonparametric tests revealed significantly lower performance for persons with mental retardation and dementia in all areas assessed. Cut-off scores were also developed for the sample in order to maximize sensitivity and specificity for the test battery. Despite the small sample size, these findings suggest that there are significant measurable differences in several neurocognitive domains between the two groups.