A 53-year-old man was referred for evaluation of decreased visual acuity over the past 2 years. He had a diagnosis of α-mannosidosis since childhood, which was confirmed by a repeated enzyme assay in his forties. He had associated cognitive impairment, musculoskeletal dystrophy, and hearing loss and was nonverbal. According to his caregiver, he previously had normal vision including normal findings on eye examinations, but during the past 2 years he had increasing difficulty with near tasks. On examination, the patient had coarse facies and evidence of prior craniosynostosis surgery and was unable to ambulate. Visual acuity was central, steady, and maintained in both eyes; pupils were equally round and reactive without an afferent pupillary defect. Confrontational visual fields were full, and intraocular pressure was 14 mm Hg in the right eye and 16 mm Hg in the left. Anterior segment examination was notable for a slight corneal haze in the right eye only but was otherwise unremarkable. Lenses showed minimal nuclear sclerosis. Fundus examination revealed slightly pale discs with a cup-disc ratio of 0.1. There was attenuation of the retinal vasculature. The posterior pole demonstrated mottled patches of the retinal pigment epithelium (RPE), most notable in the macula and surrounding the fovea. Additionally, there were numerous nummular yellow-white deposits evident at the level of the RPE (Figure 1). There was no foveal light reflex or peripapillary sparing, and there were no bone spicule pigmentary changes in either eye. Spectral-domain optical coherence tomography revealed retinal thinning, with loss of the outer retina and inner segment–outer segment junction, RPE atrophy with a corresponding highly visible choroid, and a slight epiretinal membrane (Figure 2). Fundus autofluorescence showed granular areas of hypoautofluorescence in the macula as well as in the posterior pole surrounding the optic nerve where speckled hyperautofluorescence was intermixed with hypoautofluorescent areas (Figure 3).