Congenital iris deficiency: 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 that cause 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, what tests are done for patients with congenital iris deficiency? The following is a brief description of the tests that should be done: The first step is to consider the genetic aspects and to perform the necessary genetic tests to understand what type of inheritance is present in each case of aniridia. The analysis of chromosomal bands and other genetics on the PAX-6 gene is particularly useful for the study of all lines. The following should be done: 1. A comprehensive family history, especially to know about any ocular abnormalities and/or low vision, dental abnormalities, umbilical protrusion, genitourinary abnormalities including Wilms’ tumor or mental retardation; 2. A thorough examination of the patient, including dental umbilical, genitourinary (including MRI examination) and central nervous system (including MRI examination); 3. By ocular examination, and checking their parents and other close relatives. The ocular examination must include fluorescence angiography of the anterior and posterior segments of the eye to detect abnormalities in the iris ciliary area and in the central concave avascular area despite normal appearance of the iris and macula.