The brother of case 1, a 12-year-old Jamaican boy, reported difficulty reading for 3 years. His BCVA at presentation was 20/80 OD and 20/40 OS. Fundus examination was significant for vessel sheathing in both eyes (though most prominent at the superior arcuate fibers in his left eye), central retinal pigment epithelial (RPE) changes, bilateral macular retinoschisis, and serous retinal detachments. Fluorescein angiography demonstrated multiple hyperfluorescent spots in the periphery with central leakage while indocyanine green angiography yielded significant multiple hypofluorescent spots in the periphery with hyperfluorescence centrally. Genotypic analysis of the patient's blood revealed the same mutation in BEST1 demonstrated in his sister. Also like his sister, sequencing of the entire coding region of XLRS1 in this patient also demonstrated no disease-causing variations. Follow-up at 2 years demonstrated stable visual acuity, fundus examination, and ocular coherence tomography findings of the right eye and worsening visual acuity (20/100 BCVA) with a new full-thickness macular hole in the left eye (Figure 7).