Coats disease is a developmental retinal vascular anomaly, characterized by predominantly unilateral peripheral retinal telangiectasia, nonperfusion, and aneurysmal changes with fluid and lipoprotein exudation.1 However, presentations of exudative retinal telangiectasias, often termed Coats-like retinopathies, can be observed in patients with certain systemic conditions, such as facioscapulohumeral dystrophy and telomere abnormalities.2,3 We report a novel observation of retinal vasculopathy in a patient with Leri-Weill dyschondrosteosis (LWD), a skeletal dysplasia associated with short-stature homeobox (SHOX) gene sequence variations.