Background: Arnold-Chiari malformation is defined as a congenital neuroanatomic deformity of the craniocervical junction, which is characterized by herniation of contents from the posterior cranial fossa through the foramen magnum into the cervical spinal canal. There is limited literature on audiological and vestibular evaluation on individuals with Arnold-Chiari malformation. Thus, the present study reports the results of a detailed audio-vestibular evaluation in an adult female diagnosed with Arnold-Chiari malformation.Case report: A detailed audio-vestibular evaluation was carried out in an adult female diagnosed with Arnold-Chiari malformation. Pure tone audiometry, immittance evaluation, otoacoustic emissions, auditory brainstem response (ABR), cervical vestibular evoked myogenic potential (cVEMP), ocular vestibular evoked myogenic potential (oVEMP), and video head impulse test (vHIT) were recorded, and the results were analyzed. The results of the study showed an abnormal prolongation of inter-peak latencies in the ABR, which was seen in both ears. Also, cVEMP and oVEMP was absent in both ears. The results suggest possible compression of the cerebellar tonsils on the cochlear and vestibular nuclei. Conclusions:The study suggests that individuals with Arnold-Chiari malformation should undergo a detailed audio-vestibular evaluation to understand the pathophysiology of the disorder, which can guide further management.