A 3-year-old child presented with congenital bilateral facial nerve palsy with bilateral profound sensorineural hearing loss. High Resolution Computed Tomogram (HRCT) of the temporal bones found bilateral atresia of cochlear nerve canals, incomplete partition of the cochleae and narrow facial nerve canals. Magnetic resonance imaging (MRI) revealed bilateral hypoplasia of facial nerves and aplasia of both vestibulocochlear nerves. There have been no other reported cases with this presentation. The possible aetiology and treatment options for the patient are discussed. We highlighted the review of aplasia/hypoplasia of the facial nerve and hypoplasia of cochlear nerve canal.