A 59-year-old woman with known FRDA and diabetes was referred for investigation of progressive loss of vision in the left eye. Two months previously, her visual acuities were 6/6 OU. On her first visit to the ophthalmology department,her visual acuities were 6/6 OD and 6/24 OS. Electrophysiology revealed normal rod, cone, and pattern electroretinographic results. The electro-oculographic results were normal, but the pattern reversal visual evoked potential showed increased latency in the right eye and no response in the left eye, consistent with optic nerve or optic tract disease. Clinical examination and autofluorescence imaging revealed multiple yellowish deposits at both posterior poles, consistent with pattern dystrophy (Figure). There were no signs of diabetic retinopathy. Twelve months later, her visual acuities were bilateral light perception only, with pale featureless optic discs. A computed tomographic scan of the brain and orbits excluded mass lesions or intraorbital abnormalities, and magnetic resonance imaging revealed severe atrophy of the optic nerves, optic chiasm, and optic tract.