Joubert Syndrome (JS) is a rare autosomal recessive condition presenting features of ataxia, mental-motor retardation, hypotonia, opthalmological issues and respiratory problems. The hallmark of this syndrome is ‘molar tooth sign’ in MRI due to malformations of cerebellar vermis. It is caused by mutations in genes primarily responsible for ciliary function. Therefore, multi-organ issues such as retinal, renal, oral, hepatic, digital and cerebral organs can also coexist. We present a case report of a child diagnosed with Joubert Syndrome, having symptoms of hypotonia, truncal ataxia, poor mental abilities, poor motor abilities and delayed speech and language with hearing loss. The results of audiological evaluation revealed bilateral severe to profound hearing loss. JS being one of the ciliopathy there are chances of having associated damage in sensory hair cells of cochlea resulting in significant hearing loss. Moreover, consanguinity in this case is expected to have increased the expression of autosomal recessive disorder such as JS. Since, there is dearth of literature regarding occurrence of congenital hearing loss in JS, the present case report throws light on this association. It becomes difficult to identify hearing loss when multiple disabilities coexist such as in Jouberts syndrome. Therefore, we suggest pediatricians and other health practitioners to recommend these cases for audiological screening to rule out any hearing loss at the earliest. Whereas, for JS with hearing loss an Audiologist to initiate the intervention by fitting of amplification device followed by intensive listening training and speech and language therapy to ensure a better quality of life.