BACKGROUND: Neurofibromatosis type 2 (NF2) is a rare genetic condition caused by mutations in the Merlin gene on chromosome 22. It results in acoustic neuromas (schwannomas) and other CNS tumors including meningiomas and ependymomas. Most patients develop hearing loss as a result of neuroma-driven destruction of auditory nerves. Surgery and radiation therapy remain the two most commonly recommended treatment options. However, there is a risk of further hearing loss with these procedures. There is emerging evidence that bevacizumab, a monoclonal antibody against VEGF-A, can shrink acoustic neuromas and mitigate hearing loss. CASE PRESENTATION: A 34-year-old female with bilateral acoustic neuromas from NF2 suffers partial hearing loss in the left ear and total hearing loss in the right ear after removal of the right-sided neuroma. Baseline MRI showed a left-sided acoustic neuroma (15 x 13 mm) and recurrence of the right-sided neuroma (18 x 14 mm). Bevacizumab was initiated at 5 mg/kg IV every 14 days. After 8 cycles, the patient reported marked improvement in hearing. At lower frequencies (< 1,000 Hz, the range of human voice), auditory thresholds improved by up to 60% of baseline, while at higher frequencies, improvements of up to 46% were seen. Repeat imaging showed no disease progression. CONCLUSIONS: Bevacizumab led to hearing improvement and prevention of disease progression after 8 cycles of therapy. This treatment should be considered in patients with NF2 and acoustic neuromas who wish to pursue a less-invasive treatment option with the potential of delaying progression and mitigating hearing loss.