Aims <p>To present the clinical features, presentation, diagnosis, management, and a review of outcomes for cochlear implantation done in Goldenhar syndrome (Oculo-Auriculo-Vertebral Dysplasia).</p> Materials and methods <p>A report of a child with features of Goldenhar syndrome diagnosed clinically, who also had profound SNHL with both inner and external ear anomalies and was unresponsive to hearing aids, underwent cochlear implantation without significant complications. A year of follow-up, with a review of the outcomes, is presented.</p> Results <p>Intraoperative telemetry and postoperative MAPping data are presented. At one year of postoperative follow-up, there was an improvement in CAP scores with aided audiometric responses at the lower end of the speech spectrum.</p> Conclusion <p>Cochlear implantation is a safe and reliable treatment option for children with Goldenhar syndrome without any absolute contraindications. Historically, children do well with adequate auditory-verbal therapy. However, a guarded prognosis for speech acquisition should be explained.</p>