DOI: https://doi.org/10.36347/sjams.2026.v14i04.030

Pages: 617-621

Axenfeld–Rieger syndrome (ARS) is a rare developmental disorder characterized by anterior segment dysgenesis and frequently associated with glaucoma. We report a late incidental diagnosis of ARS in a 59-year-old patient without ocular hypertension or glaucomatous optic neuropathy. The patient presented with bilateral anterior segment abnormalities, including posterior embryotoxon, iris stromal hypoplasia, and iridocorneal angle dysgenesis, associated with craniofacial anomalies and severe bilateral sensorineural hearing loss. Multimodal imaging using anterior segment optical coherence tomography and ultrasound biomicroscopy revealed significantly narrowed angles with reduced angle opening distance (AOD500), confirming structural dysgenesis. Despite these marked anatomical abnormalities, intraocular pressure and optic nerve evaluation remained within normal limits. This case illustrates the phenotypic variability of ARS and demonstrates that significant structural abnormalities may exist without glaucoma, even in late adulthood. Lifelong ophthalmologic follow-up remains essential due to the potential risk of delayed glaucoma onset.