Abstract: Goldenhar syndrome is a rare, congenital malformation syndrome characterized by dysplasia of structures driving from the first and second branchial arches. The etiology is complex and varied with the influence of genetic and environmental factors, but the specific pathogenesis is still poorly elucidated. The main manifestations are cranial and facial dysplasia, and other organs or systems may be involved. The ocular manifestations of Goldenhar syndrome are complex and diverse, involving the anterior segment, eyelid and appendages, posterior segment, extraocular muscle and orbit, among which epibulbar choristoma and upper eyelid coloboma are the most common abnormalities. Personalized treatment should be developed according to patients' conditions. Surgical treatment is the main treatment for ocular abnormalities. Early intervention is recommended for the sake of visual development and psychosocial health of patients. (Int Rev Ophthalmol, 2022, 46: 267-272)

Key words: Goldenhar syndrome, oculo-auriculo-vertebral syndrome, limbal dermoid, eyelid coloboma