AbstractGoldenhar syndrome is an uncommon disorder characterized by a variety of defects including craniofacial tissues, vertebrae, and internal organs. It preferably affects one side of the body. The origin of this illness is unknown because it is genetically variable and can be caused by a variety of factors. We present a case with Goldenhar syndrome with microtia, as well as systemic involvement, that was evaluated clinically and radiographically. The patient had many common symptoms of the illness as well as a few unusual ones. The many elements of this rare disease have been examined, with a focus on early detection and a multidisciplinary management strategy.