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. The gene discussed is FMR1; the disease is fragile X-associated tremor/ataxia syndrome.