We report a case of retinal detachment with Kniest dysplasia for which vitreous surgery was effective. The patient was a 7-year-old female who had the typical ocular and physical findings of this disease. Visual acuity was light perception alone due to a hypermature cataract and bullous retinal detachment. We performed cataract surgery and pars plana vitrectomy, and used silicone oil tamponade. After seven months, the silicone oil was removed, and visual acuity improved to 0.09. We conclude that bullous retinal detachment with Kniest dysplasia can be treated by vitreous surgery in combination with the use of silicone oil tamponade.

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