ZNF408 is a zinc finger transcription factor involved in the regulation of genes critical for retinal vascular development 1. The protein functions as a DNA-binding transcription factor with RNA polymerase II-specific activity that regulates the expression of genes essential for vasculature development 2. ZNF408 mutations cause familial exudative vitreoretinopathy (FEVR), an inherited disorder characterized by abnormal retinal angiogenesis and peripheral retinal avascularity 3. Pathogenic variants in ZNF408 are inherited in an autosomal dominant manner 3. Mechanistically, the p.His455Tyr missense mutation reduces ZNF408's DNA-binding ability and disrupts the expression of genes involved in vascular development 1. In zebrafish models, znf408 mutations cause progressive retinal vascular pathology, including deficient hyaloid vessel development, ectopic vascular hyper-sprouting, and vascular leakage 4. Clinical studies demonstrate that ZNF408 variants account for a small fraction of FEVR cases, with novel missense mutations identified in Chinese cohorts 5. While ZNF408 represents a rare genetic cause of FEVR compared to LRP5 and FZD4, its identification expands the mutational spectrum of this retinal vascular disease and confirms the critical role of transcriptional regulation in retinal angiogenesis.