Peripapillary and macular retinoschisis are usually associated with optic disc pits. We report a rare case of peripapillary retinoschisis with optic disc hypoplasia. A 59-year-old woman presented with asthenopia. Her best-corrected visual acuity was 20/20 OD. Ophthalmoscopy of the right eye revealed peripapillary retinoschisis, optic disc hypoplasia and dilated and tortuous radial peripapillary capillaries. There was no obvious optic disc pit or vitreous traction on optical coherence tomography (OCT) or fluorescein angiography. OCT showed retinoschisis around the optic disc, a thin sheet of fenestrated tissue on the optic disc and absence of serous retinal detachment. These findings had been almost the same at a previous visit to our hospital 17 years previously. Peripapillary retinoschisis may occur in patients with optic disc hypoplasia. We report a case in which visual acuity and symptoms did not change significantly after 17 years of follow-up.