El sindrome de Goldenhar es una enfermedad poco frecuente, presentandose en 1 de cada 25.000 nacidos vivos. Se caracteriza por presentar una amplia gama de signos y sintomas, principalmente sordoceguera. Se trata de una displasia oculo-auriculo-vestibular, que presenta microsomia craneofacial, quistes dermoides oculares y anomalias espinales. Se discuten las causas, afectando principalmente a varones con una relacion V/M 3:2. Abstract Goldenhar syndrome is a rare disease, 1 in 25,000 live births, which is characterized by a wide range of signs and symptoms, mainly deafblindness. This is a dysplasia oculo-auriculo- vestibular presenting craniofacial microsomia, ocular dermoid cysts and spinal abnormalities. We discuss the causes, mainly affecting males with a ratio V / M 3:2.