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Background: Revesz syndrome is a rare type of the dyskeratosis congenita spectrum disorder that is characterized by nail dystrophy, oral leukoplakia, and abnormal skin pigmentation. The retinal features are similar to those of exudative retinopathy with avascular areas of the peripheral retina. There are only a few publications describing patients with Revesz syndrome who underwent ocular treatments for the retinal complications. We report a Case of Revesz syndrome with bilateral retinal detachments that were successfully reattached by pars plana vitrectomy., Observations: A 3-year-old Japanese girl with Revesz Syndrome had progressive vitreal hemorrhages and tractional retinal detachments in both eyes. She underwent pars plana vitrectomy with lensectomy on both eyes. A retinal attachment with vision improvement was achieved by a single surgery for the right eye and after repeated surgeries for the left eye. Postoperative electroretinographic (ERG) examinations of the right eye showed a negative type ERG with the b-wave/a-wave ratio <1.0. There were extensive areas of avascular retina detected by fluorescein angiography and a thinning of the inner and outer retina detected by optical coherence tomography., Conclusion and Importance: Pars plana vitrectomy can effectively treat the extensive retinal detachment in an eye with Revesz syndrome. However, postoperative retinal ischemia can be detected by careful imaging., Competing Interests: Koh-Hei Sonoda received grants from Alcon, Novartis, HOYA, Santen Pharmaceutical, Senju Pharmaceutical, Otsuka Pharmaceutical and AMO Japan; and lecture fees from Santen Pharmaceutical, Alcon, AMO Japan, Bayer, Novartis, Kowa Pharmaceutical, Senju Pharmaceutical, Otsuka Pharmaceutical, and RE Medical. Hiroyuki Kondo received grants from Alcon, Novartis, and AMO Japan; and lecture fees from Santen Pharmaceutical, Alcon, AMO Japan, Bayer, Novartis, Kowa Pharmaceutical, Senju Pharmaceutical, Otsuka Pharmaceutical, and RE Medical., (© 2021 The Authors.)