Clinical diagnosis of fronto-temporal dementia (FTD) and its rare variants (semantic dementia and primary progressive aphasia) represents a special challenge bearing in mind its difficulties and is important due to therapeutic differences with the most common Alzheimer's dementia. Of note, cholinesterase inhibitors commonly prescribed in Alzheimer's disease are not effective in FTD and a novel drug, memantine, has not been evaluated as yet. Neuropsychological evaluation is an important add-on to neurological, psychiatric and neuroimaging assessments in the clinical diagnosis of FTD. In this paper, we have described characteristics of neuropsychological deficits observed in FTD (such as attention, language, visuospatial and memory impairments) as well as tools used (tests) in clinical practice. Special attention is paid to the utility of neuropsychological examination in the differential diagnosis of FTD versus Alzheimer's disease, vascular dementia and other, less common forms of dementia.