We recently reported a case of SOX11 (sex-determining region Y-related high-mobility-group box transcription factor 11)-related Coffin–Siris syndrome with the first observation of childhood glaucoma [17] presenting with maximal intraocular pressure of 43 mmHg, microcornea with central opacity, aniridia and cataract. The gene discussed is SOX11; the disease is isolated aniridia.