OBJECTIVE: To report two patients presenting phenotype of oculo-auriculo-vertebral (EOAV) (Goldenhar syndrome) and a history of gestational exposure to misoprostol. CASE REPORT: the first patient is a boy initially evaluated at 1 year and 8 months old. The mother had made use of misoprostol (two tablets by mouth) in the first trimester of pregnancy. This progressed to preterm labor, but no vaginal bleeding. The child had growth retardation; choroid plexus cysts right hemifacial microsomia and facial paralysis on the left, right microtia, lumbar scoliosis with hemi vertebra and hyperplasia of the right first rib, cryptorchidism, shortening of the second and third toes on the right and delay neurological development. His karyotype was normal. The second patient is a boy initially evaluated with 2 days of life. The mother had made use of misoprostol (three tablets three intravaginal and oral) in the first trimester of pregnancy. This progressed to vaginal bleeding and provision placenta. The child had hemifacial microsomia and facial paralysis on the left microtia with absent auditory canal on the left, bronchogenic cyst, atrial septal defect type patent foramen ovale, hypotonia and swallowing disorders. His karyotype was normal. CONCLUSION: In our review of the literature found no reports of association between EOAV and gestational exposure to misoprostol. However, we cannot exclude the possibility that the vascular action of the drug may be associated with the origin of the phenotype observed in patients, since disruption is one of the causes associated with the spectrum.