Less frequently, PSP can present with clinical features similar to the behavioral variant frontotemporal dementia (PSP‐FTD).3, 4, 5 Although a number of studies have reported the correlation between the clinical phenotype and the distribution and severity of tau pathology in PSP‐RS, PSP‐P, PSP‐CBS, and PSP‐PAGF,2, 6, 7, 8 given the rarity of PSP‐FTD, there are few neuropathologic studies; most of the current literature includes case reports or small case series. Here, MAPT is linked to frontotemporal dementia.