Oculopalatoskeletal syndrome is an extremely rare developmental defect characterized by the eyelid triad of blepharophimosis, blepharoptosis and epicanthus inversus, skeletal defects in the form of spina bifida occulta, craniosynostosis, cranial asymmetry, abnormality of the occipital bone, radioulnar synostosis, cleft lip and palate, hearing loss and mild mental retardation. In addition, a developmental defect of the anterior segment of the eye leading to corneal stromal opacities, and limitation of upward gaze is present in affected individuals.