A 6-year-old girl had an eye examination for the first time. Visual acuity was 0.3 in the right eye and 1.0 in the left without cycloplegia and refractive correction. In anterior segment examination of the right eye, posterior embryotoxon was observed. Posterior embryotoxon, dissociated from Schwalbe’s line in the upper half of the structure, and extending iris root fibers were present. Iris bands were laying obviously in the superioinferior direction towards the Schwalbe’s line. Because of the peripheral anterior synechiae and iris stromal atrophy, the eye was heterochromatic. Intraocular pressure was 17 mm Hg in the right eye and 13 in the left by applanation tonometry. There was no abnormality in the posterior segment in both eyes. After cycloplegia was performed, there appeared a refractive anomaly as +4.00,+0.25 α90°in the right eye and +1.00,+0.50 α85° in the left. Visual acuity increased up to 0.4 with refractive correction. The patient was examined for any systemic problem. Facial asymmetry was observed. Dental examination revealed the absence of superior and inferior molar teeth germ. There was a double navel and a skin fold on the periumblical region in the abdominal examination. No skeletal anomaly was observed. The case was considered as a unilateral Axenfeld-Rieger anomaly. After the occlusion therapy, which was performed to the left eye as 6 hours/day for 2 months, visual acuity increased up to 0.6 in the right eye. There was no family with eye and facial abnormalities.

Keywords: Axenfeld-Rieger Syndrome, posterior embryotoxon, unilateral