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Goldenhar's syndrome (oculo-auriculo-vertebral spectrum) is a craniofacial microsomy involving the first two pharyngeal arches, and must meet the morphological criteria of Feigold and Baum, which include auricular, vertebral and epibulbar dermoids. Other anomalies may be present, especially cardiac anomalies due to the possible negative prognosis, and it is relevant for pediatricians to know their prevalence. In order to carry out this review, studies on the Scielo, PubMed and VHL databases were thoroughly analyzed. Relevant studies in the area show an average frequency of cardiac malformations in patients with Goldenhar syndrome of 32%, an index that fits the great statistical variability (between 5% and 58%). The most prevalent anomalies in the face of the studies and so ordered are: conotruncal defects / outflow defects, septal defects, and other defects, with some of their subtypes well described as to the indication for cardiac surgery and risk of death in the first two years of life. The main malformation found is the tetralogy of Fallot, and among the least prevalent, but well-reported, is dextrocardia. The early evaluation of these patients is important for diagnosis and follow-up, and the echocardiographic findings associated with the clinic are of great value.

A prevalência das malformações cardíacas na Síndrome de Goldenhar (Espectro Óculo-aurículo-vertebral)