Goldenhar or oculo-auriculo-vertebral (OAV) syndrome is a congenital disorder that is usually apparent at birth. Sometimes, its diagnosis can be delayed and it is first diagnosed in adolescents or adults. We present a case of an 18-year girl who presented with chief complaint of multiple carious teeth and was found to have abnormal facies. On examination, it was found that the patient had asymmetry of face due to hypoplastic maxilla and mandible on the left side. Patient also had presence of ocular dermoid in the left eye, pre-auricular tags, scoliotic posture, ankyloglossia and fissured tongue, which were in accordance with OAV syndrome. This case report unveils the need for an early diagnosis and prompt treatment of the anomalies found in this syndrome.

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