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Visual acuity was 20/200 in the right eye and 20/30 in the left eye. Ophthalmoscopy disclosed bilateral optic nerve colobomas (Fig 1A, B) with an area of circumscribed macular pigmentary degeneration in the right eye (Fig 1A). Optical coherence tomography of the right (Fig 1C) and left (Fig 1D) eyes revealed extensive macular retinoschisis. The fundus findings together with the patient's medical history of kidney transplantation due to renal hypoplasia confirmed a diagnosis of papillorenal syndrome.