Goldenhar syndrome associated with contralateral agenesis of the internal carotid artery.

Congenital absence of the internal carotid artery (ICA) is an extremely rare vascular anomaly. Aplasia and displacement of the horizontal portion of the petrous carotid artery have been described in a patient with mandibulofacial dysostosis. To the best of our knowledge, the association between Goldenhar syndrome and ipsilateral ICA agenesis has emerged only in one case documented in the medical literature to date. We describe here a case that illustrates the association of Goldenhar syndrome with contralateral agenesis of the ICA incidentally detected on brain magnetic resonance imaging and subsequently confirmed on magnetic resonance angiography and high resolution computed tomography.