The patient was referred to the Associated Retinal Consultants, Royal Oak, Michigan, in 2004 at age 9 years. At this time, he had a 2-year history of recurrent vitreous hemorrhages in the left eye with a recent spontaneous vitreous hemorrhage in the right eye. His visual acuity was 20/30 OU. Dilated fundus examination revealed bilateral nasal dragging of his discs, absent foveal reflexes, and bilateral peripheral schisis cavities (Figure 1). A diagnosis of probable CXLRS was given, and the patient was scheduled for electroretinography as well as an examination under anesthesia with OCT. His OCT findings confirmed the presence of bilateral peripheral schisis cavities, and electroretinography showed decreased a- and b-waves. A detailed family history revealed a maternal grandfather diagnosed as having retinitis pigmentosa, which likely represents a misdiagnosed case of CXLRS. Unfortunately, it was not possible to examine the patient's grandfather to confirm or disprove this theory. The patient was followed up at regular 6-month intervals with serial clinical examinations and OCT scans. Vision and funduscopic examination findings remained stable. In 2010, his peripheral blood was screened for mutations in RS1. This test showed a positive missense mutation, R102Q (CGG to CAG), on exon 4 of RS1. At his most recent follow-up visit in October 2010, his visual acuity was 20/25−1 OD and 20/20+1 OS. Spectral-domain OCT showed redemonstration of peripheral schisis cavities but failed to show any detectable evidence of the foveal cystic changes that have been considered universal in CXLRS (Figure 2). Central foveal thickness was normal at 266 μm OD and 276 μm OS. A return visit was scheduled for 6 months later.