Axenfeld-Rieger syndrome encompasses systemic abnormalities such as dental, cardiac, craniofacial, and abdominal-wall defects. As a rare autosomal dominant condition, it presents as a heterogeneous group of features. Managing this syndrome requires a multidisciplinary approach involving dentists, orthodontists, oral surgeons, and geneticists due to the complexity of dental anomalies and the rarity of the condition. Early diagnosis is pivotal not only for addressing dentofacial appearance and function but also for detecting systemic abnormalities that could impact vision. Collaboration among specialists, including ophthalmologists and maxillofacial surgeons, is vital for comprehensive care. Diagnosis is typically based on clinical and ophthalmologic examinations, supplemented by genetic analysis if the syndrome is suspected. Treatment plans are customized by specialized teams to meet the distinct needs of each patient.