The osteoporosis-pseudoglioma syndrome (OPPG) is a rare autosomal recessive disorder characterized by severe juvenile-onset osteoporosis, which ranges between mild to moderate, and congenital or early-onset blindness occurring due to hyperplasia of the vitreous, to corneal capacity, and to secondary glaucoma. Other manifestations include muscular hypotonia, ligamentous laxity, mild mental retardation and seizures. Prevalence of OPPG is estimated at 1 per 2,000,000 and the age of onset mainly occurs during childhood.