The neuropathology of frontal lobe degeneration of non-Alzheimer type was reevaluated on the basis of a new material of 13 cases against the background of experiences from earlier published 16 cases. The salient neuropathological feature was an unspecific neuronal degeneration of superficial cortical layers of the frontal and to some extent the temporal lobes without markers for Alzheimer''s, Pick''s or Lewy body diseases and there were no indications so far of a prion etiology. The consistency of histopathological features are taken to indicate a disease entity, also identified by other authors. It is tentatively grouped together with progressive and aphasic dementia, similar cases in the literature and possibly also Pick''s disease and ALS with dementia.