1. An autopsy case of frontotemporal dementia with onset at the early age of 28 years is reported. 2. The neuropathological features consisted of limited, knife-like frontotemporal atrophy with severe neuronal loss, spongiform change and gliosis, which is compatible with the frontotemporal dementia. 3. Biochemical determinations disclosed that biogenic amines and their metabolites, predominant in the dopaminergic markers, were depleted in the damaged regions. 4. Since biochemical data in frontotemporal dementia are few in previous studies, it will be determined whether these biochemical changes are characteristic for the juvenile type of frontotemporal dementia or not.