A 19-year-old man with Alport syndrome presented with diminution of vision in his left eye. Slit-lamp biomicroscopy showed an early anterior lenticonus with an anterior subcapsular cataract in his right eye (Fig. 1A) and a dense anterior polar and anterior subcapsular cataract in his left eye (Fig. 1B). Fundus photography revealed central perimacular dot-and-fleck retinopathy with a dull macular reflex or lozenge in both eyes (Fig. 1C, 1D). The flecks were better visualized on red-free imaging as a ring of fine white stippling around the macula (Fig.