Bilateral Anterior Segment Dysgenesis (BASD) encompasses a group of rare congenital anomalies involving abnormal development of the cornea, iris, lens, and anterior chamber angle. These conditions often result in visual impairment, glaucoma, and associated systemic abnormalities. This article explores specific syndromes linked to BASD, including Axenfeld-Rieger syndrome, Peter’s anomaly, aniridia, and ICE syndrome. It provides an updated perspective on clinical features, underlying mechanisms, diagnostic approaches, and management strategies to enhance recognition and treatment of this complex condition.