AIM: To describe the stomatognathic systems and audiological features in 2 children with Goldenhar syndrome. CASE REPORT: Oculo-auricular-vertebral dysplasia (Goldenhar syndrome) is characterized by a triad of ocular, auricular, and vertebral diseases. It is a rare congenital syndrome and is multifactorial, heterogeneous, and phenotypically very variable. The incidence is 1 in every 5.600-26.500 live births, with a male:female ratio of 3:2. The etiology is still unknown, but the condition is believed to result from changes in the formation of structures arising from the first and second branchial arches. Major auricular changes observed in patients with Goldenhar syndrome are microtia/anotia, auricular appendices, and complete absence of the pavilion and the external acoustic meatus. In the eyes, the presence of epibulbar dermoids and microphthalmia is reported. Along with facial asymmetry, vertebral, pulmonary, and cardiovascular diseases are found. We evaluated hearing and stomatognathic systems in 2 children with Goldenhar syndrome and compared the findings with alterations reported in the literature on patients with this syndrome. Both patients had cleft palate at birth, auricular malformations with hearing loss in at least one ear, and changes in stomatognathic function (e.g., deglutition). To address the physical, mental, and social welfare of these patients, the speech therapist works on development of the impaired skills, making it necessary to intensify research that will contribute to the continued enhancement of speech therapy for future cases. CONCLUSION: Although these patients have the same disease, the manifestations of Goldenhar syndrome differ, and each patient requires individualized therapeutic care.