BACKGROUND: Neuroectodermal syndromes are complex because of their rarity and overlapping clinical manifestations making differential diagnosis problematic. CASE REPORT: A 2-year old girl presented with a phenotype characterized by bilateral pre-auricular tags, a fistula of the right cheek, hemifacial microsomia, and a limbus dermoid on the right eye. This constellation is characteristic for the Goldenhar syndrome. Following excision of the tags and fistula along with a keratoplasty, the child developed normally. CONCLUSION: The exact diagnosis of a neuroectodermal syndrome facilitates identification of associated symptoms. Early surgical therapy may prevent the development of functional deficits.