Retinal detachment (RD) repair in patients with X-linked retinoschisis presents technical challenges due to the relative fragility of the retina and separation of the retinal layers. We present the case of a 10-year-old boy, molecularly confirmed hemizygous mutation of the RS1 gene, who first presented as a 1-year-old with bilateral bullous retinoschisis involving the macula. The bullous retinoschisis improved with carbonic anhydrase inhibitor (CAI) eyedrops, and patient remained stable for 8 years. He subsequently developed a left vitreous hemorrhage and RD with vitreous traction and preretinal proliferation. He underwent a standard 3-port 23-gauge pars plana vitrectomy, membrane peel and air-fluid exchange with good anatomical outcome and visual improvement. We highlight the importance of differentiating the different layers—posterior hyaloid, the inner retinal layer and outer retinal layers—during the vitrectomy to achieve anatomical success.