Congenital aniridia is an autosomal dominant condition, mostly bilateral, in which the iris tissue is not visible on clinical examination and the equatorial part of the lens and the suspensory ligament are exposed. Patients often have severe photophobia and often have nystagmus and low vision because they are accompanied by other ocular developmental abnormalities, such as congenital cataracts and macular hypoplasia. Iris and/or choroidal defects are due to incomplete closure of the fissure below the optic cup during embryonic development, and some patients may have a partial defect of the optic nerve. In choroidal defects, a large area of sclera is revealed starting below the optic papilla, and retinal vessels located on its surface are visible. The edges of the defect area are often surrounded by irregular pigment. It may be accompanied by congenital anomalies such as microphthalmia, microcornea, and nystagmus, with varying degrees of visual impairment. Congenital pupillary remnants Congenital pupillary remnants are often filamentous, cord-like, or arachnoid-like, crossing the pupil from one side of the iris convoluted wheel to attach to the opposite side of the iris convoluted wheel. Unlike post-inflammatory post-iris adhesions, they usually do not affect vision and pupil movement is normal. No treatment is required. Occasionally, surgical excision should be considered for those with large remnants of membrane covering the surface of the lens and affecting vision.