PURPOSE: To report the ocular findings in two siblings with IFAP and their 
mother and to review the natural course of the keratopathy of this disease.
METHODS: Clinical ophthalmological examination of all patients and fundus 
photography of the carrier mother were performed.
RESULTS: Both affected male children had severe photophobia, total superficial 
and deep corneal vascularization, and reduction of vision to counting 
fingers.The mother had tortuous retinal vessels.
CONCLUSIONS: Males with IFAP have an inexorable progression of corneal 
vascularization and loss of vision. Retinal vascular tortuosity may be another 
clinical sign of carrier status in females.