The authors present a case of Goldenhar syndrome, with its characteristic features and variations. A male child of four years old at the Ophthalmology Service, Hospital São José The diagnosis was completed due to external and internal changes of the patient, after being evaluated by pediatrics, genetics, otolaryngology and ophthalmology.The oculoauriculovertebral spectrum (OAVS) known as Goldenhar Syndrome is a rare, complex and phenotypically variable condition, of still unknown origin is characterized by dermoid cysts epibulbar, auricular appendices and mandibular hypoplasia. […]