Goldenhar syndrome is a congenital abnormality with an incidence of 1 in 5,200 to 26,500 births. This syndrome is characterized by facial asymmetry, ear malformation, and/or defects in the eyes and vertebrae. The hearing disorder manifests as both conductive or sensorineural due to the abnormalities occurring in the inner and outer ear. We report a case of a 1-year-3-month-old child presenting with left anotia and right microtia, severe bilateral conductive hearing loss, and global delayed development. The patient was also found to have a hemifacial microsomia, a secundum atrial septal defect (ASD), and a ventricular septal defect (VSD). The patient was advised to use hearing aids and participate in speech therapy. The management of this Goldenhar syndrome patient should be done comprehensively, appropriate to the abnormalities found to achieve the best result.