A 15-year-old boy came to the eye clinic with reduced vision in the left eye of a year's duration and prior trauma. Best-corrected visual acuity was 6/9 and hand movement in both eyes, respectively. The anterior segment examination was essentially normal except for a Marcus Gunn pupil and a polar cataract in the left eye. Goldmann applanation tonometry was 10 and 06 mmHg, respectively, in the right and left eyes. Binocular indirect ophthalmoscopy of the right eye revealed pink disc, normal vessels and the Mizuo–Nakamura phenomenon with a cartwheel appearance at the macula. The left eye had a total retinal detachment with proliferative vitreoretinopathy and retinal tear at 12 o' clock. Optical coherence tomography revealed posterior vitreous detachment and schitic cavities at the macula in the left eye. A diagnosis of left rhegmatogenous retinal detachment with background X-linked juvenile retinoschisis was made. The patient was advised on a pars plana vitrectomy under guarded visual prognosis.