A 56-year-old woman presented with decreased visual acuity in the right eye. The corrected visual acuity was 20/100 OU. On examination, both eyes revealed mild cataracts, perivenous bone-spicule pigmentation, and chorioretinal atrophy in the peripapillary and perivenous areas (Figure). Optical coherence tomography showed a macular hole in the right eye (Figure, A) and severe atrophied retinal nerve fiber layer (RNFL) in both eyes. Electroretinography revealed decreased photopic and scotopic responses, and fluorescein angiography showed diffuse window defects and blocked fluorescence. These findings indicated a diagnosis of pigmented paravenous chorioretinal atrophy (PPCRA) in both eyes combined with macular hole in the right eye. PPCRA was first reported in 1937.1 Visual function is usually good in these patients unless the macula is involved. The visual impairment in this patient can be attributed to severe atrophied RNFL. To our knowledge, lamellar,2 but not full-thickness, macular holes have been reported in PPCRA. The patient agreed to undergo a pars plana vitrectomy, after which the macular hole closed. The patient’s final best-corrected visual acuity was approximately 20/67 OD.