This case documents the co-occurrence of the fragile X-associated tremor ataxia syndrome (FXTAS) and Alzheimer-type neuropathology in a 71-year-old premutation carrier with 85 CGG repeats in the fragile X mental retardation 1 (<i>FMR1)</i> gene, in addition to an apolipoprotein E (<i>APOE)</i> ε4 allele. Here, APOE is linked to fragile X-associated tremor/ataxia syndrome.