Abstract: Klippel-Feil syndrome (KFS) is a congenital anatomical defect that occurs due to the failure of fusion of two or more cervical vertebrae in the neck region. The physical features of the condition include a short neck, low hairline at the back of the head, and restricted movement of the upper spine. Otologic anomalies have been reported to be seen in 60% of patients with Klippel Feil syndrome which includes unilateral, bilateral and external, middle & inner ear pathologies. This case study focuses on a 4-year-old female child diagnosed with Klippel-Feil syndrome presenting with bilateral hearing impairment, combined vestibulo-cochlear-semicircular canal dysplasia along with delayed speech and language development. A detailed audiological evaluation was done and the results are further discussed.