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X-linked juvenile retinochisis (XLRS) is a rare inherited bilateral vitreoretinal dystrophy which usually affects males early in life. We describe the clinical findings, outcome, and challenges in treatment of three siblings diagnosed with XLRS. Three siblings with ages ranging from 5 to 9 years old presented with reduced visual acuity (VA) and posterior segment showing varying degrees of vitreous veil and spoke-wheel maculopathy. Optical coherence tomography (OCT) of the macula was performed, revealing retinoschisis in all eyes. All three siblings were diagnosed with XLRS and were started on topical brinzolamide twice daily. OCT was repeated at 6 months and 18 months. At 18 months, three eyes showed stable VA and three eyes showed improved in VA. One out of the three eyes with stable VA showed improved retinoschisis while the other two eyes showed worsening retinoschisis. On the other hand, one out of the three eyes with improved VA had improved retinoschisis and the other two had worsening retinoschisis. We demonstrated that the VA of patients with retinoschisis is not directly proportional to the degree of splitting of the neurosensory retina. Retinoschisis treatment is challenging, as there is no one proven effective treatment up to date.