Congenital iris deficiency: complete absence of the iris, congenital absence of the iris is a developmental disorder of both eyes that can be associated with a variety of ocular disorders such as corneal opacity, microcornea, lens dislocation, cataract, glaucomatous macular dysplasia strabismus nystagmus, etc., involving the whole eye. Some comorbidities are present at birth, while others may be delayed until childhood or early adulthood. Some patients may have systemic abnormalities. So, how do you differentially diagnose congenital iris deficiency? The following is a brief description: The diagnosis can be made clinically. The diagnosis of this congenital anomaly is easy to make clinically. In combination with ocular and systemic anomalies, there are four types of iris deficiency: 1) with nystagmus, corneal opacity, glaucoma, and reduced vision; 2) with significant iris deficiency but good vision; 3) with Wilms’ tumor (iris-free Wilms’ tumor syndrome) or other genitourinary abnormalities; and 4) with mental retardation. In congenital iris-deficient membrane eyes, there is often a residual iris of varying width at the root of the iris, which can also be associated with a variety of ocular diseases. Because the degree of iris deficiency varies, so the clinical manifestations vary, but all have photophobia, frowning and squinting and other manifestations of poor vision and may progressively diminish in relation to iris deficiency, macular central hypoplasia, corneal opacification, cataract glaucoma and refractive error.