Alzheimer's disease and Pick's disease are representative dementias. Cases which do not fit prototypes are termed unclassifiable dementias. New dementia subtypes are identified when a conglomerate of clinical, radiologic and pathologic findings are consistently identified. One such variant is diffuse neurofibrillary tangles with calcification (DNTC), which has been reported almost exclusively from Japan. Significant pathological advances in this decade have established DNTC as a distinct entity. Although initially the diagnosis was neuropathologic, increasing knowledge about DNTC has made it possible for a clinical diagnosis to be made. We report a clinical case of DNTC in a Caucasian American. The diagnosis of DNTC was based on his atypical senile dementia, anomia, apathy and parkinsonian features, normal serum biochemistry, and evidence of basal ganglia and cerebellar calcification with predominant temporal lobe atrophy on neuroimaging. To the best of our knowledge, this is the first clinical description of DNTC from the United States. © 2008 Sage Publications.