Abstract
Goldenhar syndrome or oculoauriculovertebral spectrum is a rare congenital anomaly usually occurs unilaterally affecting craniofacial structures, vertebrae and other internal organs. Congenital heart defects (CHDs) have been reported in 5–58% of the patients. Here we report a case of Goldenhar syndrome with classical signs and symptoms of ocular and auricular involvement associated with Atrial Septal Defect in a school going female child, who came for the routine eye checkup which was later on confirmed clinically and by echocardiography.