[Keratotorus in Norrie disease].

We report on a 46-year old white male with Norrie disease. In the right eye he had a keratotorus with non vascularized corneal scars and mature cataract. After penetrating keratoplasty a pseudoglioma with irreversible total retinal detachment was found. The left eye had phthisis bulbi with corneal pannus and band keratopathy, shallow anterior chamber, posterior synechia and mature cataract.