A 10-year-old boy with kidney hypoplasia and chronic kidney failure presented with visual acuity of 20/25 OD and 20/40 OS. His family history was noncontributory. An examination and spectral-domain optical coherence tomography imaging revealed bilateral macular retinoschisis, subretinal fluid, and optic nerve dysplasia (Figure), consistent with papillorenal syndrome. He was referred for genetic counseling.