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Introduction: Goldenhar syndrome is a rare congenital disorder which manifests as ocular, auricular, dental and vertebral and several other systemic abnormalities. The abnormalities in the organs developing from first and second branchial arches during blastogenesis cause morphological anomalies in this disorder. Case Report: We report a case of Goldenhar syndrome in a 12-year-old Asian girl with limbal dermoid cyst, lateral canthal tag, preauricular appendage and malocclusion of teeth. Computed tomography scan and grayscale ultrasonography of orbit revealed dermoid cysts. Multidisciplinary treatment approach was advised; dermoid cyst was excised surgically and dental consultation was advised for malocclusion. Conclusion: This case has been presented to increase the awareness about this rare entity, to highlight the importance of typical clinical and radiological findings and its association with various systemic conditions. Multidisciplinary treatment approach and longterm regular follow-up is important to monitor the growth and development of patients.

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