Fragile X-associated tremor/ataxia syndrome (FXTAS) is caused by FMR1 premutation,[1] typically presenting initially with tremor, while ataxia or cognitive impairment generally manifests later.[2,3] The disorder exhibits significant clinical heterogeneity, posing diagnostic challenges when cognitive impairment is the initial presentation in middle-aged and older males. Here, FMR1 is linked to Cognitive impairment.