Goldenhar syndrome is a rare and sporadic
condition of unknown etiology characterized by a
combination of anomalies: oculo-auriculo-vertebral
(OAV) dysplasia, auricular appendices, dermal
epibulbar cysts and malformation of the ears.Here, we
report an unusual case of Goldenhar syndrome with skin
lesion suggestive of xeroderma pigmentosa.Patient with
Goldenhar syndrome has inconsistent presentation and
can affect the various parts of the body.Multidisciplinary
approach is mandatory for early diagnosis and
managementto avoid from severe complications in later
stage of life.
Keywords : Goldenhar syndrome, Oculoauriculovertebral, Preauricular tags, Vertebrae.