Currently, the clinical diagnostic criteria of frontotemporal lobar degeneration (FTLD) and dementia with Lewy bodies (DLB) are well known to neurologists and psychiatrists. However, the accuracy of the clinical diagnosis of these diseases in autopsy series is not always adequate. For example, FTLD is a syndrome rather than a clinicopathological disease entity that is comprised of various pathological substrates, including Pick's disease, FTLD with microtubule-associated protein tau gene mutation, FTLD with tau-negative ubiquitin-positive inclusions (FTLD-U), FTLD-U with progranulin gene mutation, corticobasal degeneration, basophilic inclusion body disease, and neuronal intermediate filament inclusion disease. Whether these underlying pathologies can be identified clinically is one of the greatest interests in neuropathological research. The pathophysiological relationship between Lewy pathology and Alzheimer pathology in DLB is explored with interest because it may be associated with the accuracy of clinical diagnoses. For example, although Lewy pathology may progress from the brain stem nuclei to the cerebral cortex in Parkinson's disease, recent studies have demonstrated that the progression pattern in DLB is not always identical to that in Parkinson's disease. It is also considered that the progression pattern of Lewy pathology correlates with the evolution of clinical symptoms and that the progression pattern of Lewy pathology may be altered when Alzheimer pathology coexists. In the present paper, the clinicopathological features of two demented cases are presented, and some pathological issues associated with the clinical diagnosis of FTLD and DLB are discussed.