Patients with leukemia have an increased risk of developing sensorineural hearing loss. This is a retrospective review of a profoundly deafened patient with acute myelogenous leukemia who underwent cochlear implantation. The 26-year-old patient was successfully implanted with a Nucleus cochlear implant in the complete remission after peripheral blood stem cell transplantation. To date, with a follow-up of 1 year, the patient has not experienced any complication and has regained useful open-set speech perception. To our knowledge, this is the first reported case of successful cochlear implantation in a patient deafened by acute myelogenous leukemia.