A 64-year-old man progressively developed ataxic gait, 3-Hz high-amplitude, intentional/postural tremor, and dysarthric speech. FMR1 gene analysis revealed a premutation (89 CGG repeats) and a brain MRI showed mesencephalon atrophy with normal pons morphometry (the so-called “hummingbird” or “penguin” sign) along with middle cerebral peduncle hyperintensity, the radiological hallmark of fragile X-associated tremor/ ataxia syndrome (FXTAS). The disease achieved mild severity in 2 years: Neither psychiatric symptoms nor dementia or impairment in daily living activities were reported.