Goldenhar syndrome is a rare congenital disorder characterized by defects in the development of structures derived from the first and the second branchial arches. This condition encompasses a range of symptoms, including craniofacial, ocular, vertebral, and auricular abnormalities. We present the case of a 6-year-old girl with right temporal bone hypoplasia and preauricular tag from birth, leading to a diagnosis of Goldenhar syndrome. She exhibited various middle and external ear defects, and her audiological treatment was crucial in ensuring optimal neurological and speech development. In adolescence, if the Eustachian tube remains stable, surgical repair of the ossicular chain may be considered.
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