Goldenhar syndrome is also known as oculo–auriculo–vertebral dysplasia with hemifacial microsomia which is a rare congenital anomaly involving the first and second branchial arches.The syndrome was first described in 1952 by the French ophthalmologist Maurice Goldenhar.The incidence of Goldenhar syndrome has been reported to be between 1:3500 and 1:5600, with a male:female ratio of 3:2. The exact etiology is not known .Most of the cases have been noted as sporadic. Ingestion of drugs such as thalidomide, retinoic acid, tamoxifen, and cocaine by the pregnant mother may be related to the development of this syndrome.