Auditory brainstem implantation (ABI) is the only solution to restore hearing when cochlear nerves are disrupted together with the pathologies where bilateral cochleae do not provide a suitable location for cochlear implantation. We reported first two successful auditory brainstem implantation cases in patients with neurofibromatosis Type II (NF2) with bilateral acoustic neuroma causing bilateral profound sensorineural hearing loss in Malaysia. A good candidate selection, dedicated surgeons and rehabilitation team as well as strong family support are the crucial factors in achieving the best possible surgical, audiological and speech outcomes.