Adaptive-optics scanning light ophthalmoscopy imaging revealed a small focal hyporeflective lesion in both eyes at the foveal center in the subject with RGS9-associated retinopathy, which also correlated with a decreased cell number at this location, as well as bilateral focal interruption of the interdigitation zone and inner segment ellipsoid bands on spectral-domain optical coherence tomography. Here, RGS9 is linked to retinal disorder.