A 47-year-old woman was referred to our department for gradual progression of visual impairment, including constriction of the visual field, night blindness, and photophobia. The patient, who had initially noticed poor night vision during childhood, was clinically diagnosed with fundus albipunctatus a few years earlier by a local ophthalmologist; this diagnosis was then confirmed by direct genomic sequencing, showing a homozygous Gly35Ser mutation of the RDH5 gene. The patient signed a comprehensive consent form according to good clinical practice guidelines before proceeding with any examinations. Best-corrected visual acuity was 20/80 OU. Fundus examination revealed numerous yellow-white flecks throughout the midperipheral to far-peripheral retina, and the maculae each showed a bull’s-eye lesion (Figure 1). Fundus autofluorescence showed an abnormal circular hypoautofluorescence within the macular area surrounded by a hyperautofluorescent halo in both eyes (Figure 1). The standard flash electroretinogram disclosed severely decreased a- and b-waves in both eyes after 30 minutes of dark adaptation. After 2 hours of dark adaptation, standard flash electroretinogram a- and b-waves showed recovery, although not to the normal level. The photopic electroretinogram and multifocal electroretinogram responses were significantly reduced. High-definition spectral-domain OCT (OCT 4000 Cirrus; Humphrey-Zeiss, San Leandro, California) showed reduction of central macular thickness in both eyes and allowed for visualization of transverse PR loss in the foveal region as well as disruption and focal loss of the inner segment–outer segment junction (Figure 2). Moreover, in correspondence with retinal flecks, we observed hyperreflective lesions seen as dome-shaped deposits located in the inner part of the RPE layer and at the level of the outer segment of the PR continuous with the RPE layer (Figure 2).