Abstract

Goldenhar syndrome also known as oculoauriculovertebral dysplasia is a hereditary developmental disorder characterized by classic triad with mandibular hypoplasia (facial asymmetry), ear malformation(preauricular fistula) and eyes(bulbar dermoid cyst, microphthalmia), as well as vertebral anomalies.
An 11 year old girl came with C/O mass in the right eye since birth which was single, globular, over the limbus covering 2/3rd of cornea containing hair follicles over the surface, painless and progressively increasing S/O limbal dermoid cyst associated with diminution of vision since 3 years. On examination, hemifacial microsomia,pre auricular tags, scoliosis were present. Dermoid excision under IV sedation and genetic counselling were done.