Purpose: To present the natural course and describe the characteristic findings of a case of unilateral retinal pigment epithelium dysgenesis (URPED) and highlight the optical coherence tomography angiography characteristics of this rare fundus pattern.
Methods: Case report.
Results: A 52-year-old male was referred 8 years ago to our clinic due to a distinctive unilateral lesion in his left fundus, of which he was aware from early adulthood.
A 6-year-old boy presented with unexplained unilateral low visual acuity. Best-corrected visual acuity was counting fingers in the right eye and 20/20 in the left eye. Fundus examination of the right eye revealed intermediate uveitis, with moderate vitritis, snowballs, and snowbanking.
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