The paper presents five cases of the Syndrome described by Goldenhar, which consists in oculo-auriculo-vertebral alterations. Classic descriptions relate the presence of epibulbar dermoid, pre-auricular appendixes, mandible hypoplasia and vertebral anomalies. Excepting by some vertebral alterations, all the other anomalies were common to the five cases. This syndrome may be accompanied by congenital cardiopathy in a percentage varying from 15 to 50%, depending on the publications. The reason of hospitalization was the real presence of congenital cardiopathy of important clinical repercussion. Three patients had Tetrad of Fallot, one transposition of the great arteries, and the last one total annomalous pulmonary venous connection, which died due to clinical complications. The four patients were submitted to surgical correction of their cardiac defects, being the face defects programmed to the corrected later on.