Goldenhar's syndrome, also known as oculo-auriculo-vertebral (OAV) spectrum, is a rare condition described initially in the early 1950's by Maurice Goldenhar. It is a complex syndrome characterized by an association of maxillomandibular hypoplasia, deformity of the ear, ocular dermoid and vertebral anomalies. The etiology of this rare disease is not fully understood, as it has shown itself variable genetically and of unclear causes. Here, we describe a 13-year-old male patient with classical signs of the syndrome as pre-auricular tags, microtia, kyphoscoliosis, polydactyly and cleft lip.