OBJECTIVE: To report two pairs of monozygotic twins discordant for the oculo-auriculo-vertebral (EOAV) (Goldenhar syndrome). CASE REPORT: the first pair of twins was the product of a pregnancy whose mother had used alcohol and intranasal cocaine. Both were born by cesarean section, and it was found that pregnancy was monochorionic and monoamniotic. The evaluation of the placenta showed a communication vascular site of insertion of the strings. The first twin was acardic. The second twin presented findings of EOAV: left facial microsomia and microtia on the left. His karyotype was normal. The second pair of twins was the product of a pregnancy who presented with hypertension (chronic) and gestational diabetes. The sleep graphic evaluations performed at the beginning of the second quarter, showed that pregnancy was monochorionic and diamniotic, a finding confirmed after birth. The twin EOAV carrier (1 twin) evolved with an asymmetrical restriction of growth, and it was found, soon after birth, an increased strength of its umbilical artery. The evaluation showed that after birth the fetus with EOAV had left facial smaller than the right, preauricular tags, epidermis tumor in the left eye, and abnormalities of the vertebrae and ribs. His karyotype was normal, and their evaluation for micro deletion 22q11 by FISH technique. CONCLUSION: Our findings, in agreement with some literature, and point to an important vascular component in the etiology of EOAV, which may be linked to their own conditions in twin pregnancy.