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To evaluate the efficacy of intravenous immunoglobulin in the treatment of cystoid macular edema and uveitis caused by juvenile retinitis pigmentosa in a woman interested in planning a pregnancy.Descriptive case report. A 25-year-old woman with retinitis pigmentosa presented with decreased visual acuity, interested in a second opinion.Intravenous immunoglobulin therapy was begun at 2-week intervals. Visual acuity improved from 20/100 to 20/70 by 6 weeks, where it stabilized for the remaining year. Serial optical coherence tomography imaging revealed marked improvement in cystoid macular edema after 2 treatments of intravenous immunoglobulin at 2 weeks of follow-up, with complete resolution noted at 4 months, a finding that persisted at 1 year of follow-up.Our findings support the use of intravenous immunoglobulin as a therapeutic option for the resolution of cystoid macular edema and management of uveitis secondary to juvenile retinitis pigmentosa.