We present the first case of rapid onset high myopia in early childhood as the presenting feature of Loeys-Dietz syndrome. The patient progressed from a normal degree of hyperopia (+2 diopter sphere [DS]) at 18 months of age to high myopia (-10 DS) 9 months later. Subsequent genetic testing revealed both her brothers and her father to carry the same TGFB3 pathogenic variant. This case aims to highlight the importance of considering systemic conditions in children with high myopia.
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