International Journal of Contemporary Pediatrics | May-June 2017 | Vol 4 | Issue 3 Page 1115 International Journal of Contemporary Pediatrics Jaiswal A et al. Int J Contemp Pediatr. 2017 May;4(3):1115-1117 http://www.ijpediatrics.com pISSN 2349-3283 | eISSN 2349-3291 Case Report A variant of Goldenhar syndrome Akanksha Jaiswal, Thumjaa Annamalai*, Nirupama M., Shafath Ahmed M., Ramya V. INTRODUCTION Facioauriculovertebral syndrome was first recorded by German physician, Carl Ferdinand Von Arlt. 1 Goldenhar syndrome (oculoauriculovertebral dysplasia with hemifacialmicrosomia) is a rare congenital anomaly involving the first and second brachial arches. 2 It is also known as the oculoauriculovertebral syndrome (OAVS) because of the association of eye anomalies with or without vertebral and ear anomalies. 3 Oculoauriculovertebral dysplasia, consisting of preauricular appendages, fistulas and epibulbardermoids, was first described in 1952 by the Swiss ophthalmologist Maurice Goldenhar. Gorlin et al, included vertebral anomalies as signs of the syndrome and suggested the name Oculoauriculovertebral (OAV) dysplasia for this condition. Smith used the term facio-auriculo-vertebral sequence to include both Goldenhar syndrome and hemifacialmicrosomia. The classic triad is mandibular hypoplasia resulting in facial asymmetry, ear and/or eye malformation and vertebral anomalies. Goldenhar’s syndrome is often characterized by the combination of anomalies including dermal epibulbar appendices and malformation of the ears. We present a case of a variant of Goldenhar Syndrome. CASE REPORT Figure 1: Right side facial macrosomia, right facial asymmetry with deviation of angle of mouth to the left side. ABSTRACT We report a case of 10 months old child with a variant of Goldenhar syndrome in the form of microtia of right ear, microsomia of the right-side face, right facial nerve palsy with cardiac anomaly, renal anomaly and sensorineural hearing loss on the right side which were rare associations prompted to report this case. Keywords: Clinical manifestations, Goldenhar syndrome Department of Pediatrics, Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu, India Received: 20 February 2017 Accepted: 27 March 2017 *Correspondence: Dr. Thumjaa Annamalai, E-mail: thumjaa@gmail.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20171740