Congenital iris deficiency is a complete absence of the iris, with direct visualization of the equatorial rim of the lens, suspensory ligament, and ciliary process. There may be photophobia and various ocular abnormalities causing low vision, and more patients become blind due to progressive corneal and lens clouding or glaucoma. To reduce photophobia, tinted glasses or corneal contact lenses may be worn. So, how can congenital iris deficiency be prevented? The following is a brief description of prevention methods: Clinical genetic counseling and prenatal diagnosis are required, which can minimize the recurrence of new cases in the family of the first witness, prevent the birth of genetically affected children, and improve the eugenics rate. The use of artificial iris corneal contact lenses has the effect of reducing photophobia and increasing depth of field, with other ocular congenital anomalies need to be treated accordingly, such as with cataract can be performed ultrasound emulsification and with iris septal IOL implantation, targeting PAX6 gene therapy still need further research. The incidence of congenital aniridia is a rare but serious congenital anomaly, mostly autosomal dominant, characterized by a complete iris defect with multiple congenital abnormalities of the eye, first reported by Barrata in 1819, with an incidence of about 1 in 50,000 to 1 in 100,000. It is called partial aniridia if some iris tissue is visible to the naked eye in the anterior chamber, or aniridia if only a few iris stumps are visible with atrial angioscopy. This disease is usually bilateral and has a significant impact on vision. The main cause of vision loss is amblyopia due to macular central sulcus hypoplasia, but it may also be caused by combined glaucoma, cataract and corneal clouding.