Background Kawasaki disease (KD) is an acute febrile, usually self-limiting illness of infancy and childhood, that can show audiological manifestation of sensorineural hearing loss (SNHL, 36% of patients), usually bilateral in nature. Case presentation We report a 12-year-old female diagnosed with KD at 6 years, showing atypical manifestation of asymmetrical SNHL since 1.5 years after the episode of febrile illness. The patient was administered Immune Globulin intravenously and aspirin, which were tapered to a low dose. The patient later reported reduced hearing sensitivity, ear pain, blocking in both ears, and tinnitus (intermittent) in the left ear. The audio-vestibular manifestations in the case using test battery approach (Pure tone audiometry, speech audiometry, tympanometry, reflexometry, otoacoustic emissions, auditory brainstem response, and behavioral and objective vestibular tests) is discussed. The rehabilitative measures taken, and the need for sensitization of school teachers for early identification of hearing loss in children with KD is highlighted. Conclusions This case presentation concentrates on the rare asymmetrical SNHL and vestibular finding post-KD and emphasizes the need for health care professionals to refer patients with KD to an audiologist to trace the possible sequelae including hearing loss and vestibular disorders. The atypical manifestations of KD in hearing domain is suggestive of the need for early identification, adequate profiling of not just hearing but also balance-related manifestations, instigation of appropriate rehabilitative measures, effective counselling of parents and school teachers to facilitate better academic learning, and communication opportunities.