The inborn aniridia,which is characterized by its hypoplasia of iris,is a rare and severe disease in ophthalmology.It usually accompanies other abnormalities,such as corneal opacity,cataract,glaucoma,anopsia and so on.And it is usually a hereditary disorder of chromosomal abnormality.In this article,we report two families with inborn aniridia,and review the etiological factor,clinical manifestation,therapy and prophylaxis of the aniridia.