Recurrent respiratory papillomatosis can be a devastating condition for a child, with severe consequences. Currently, there is no proven successful medical treatment. We describe the use of systemic bevacizumab to treat two children affected by aggressive recurrent respiratory papillomatosis. Respiratory symptoms and quality of life improved dramatically in both patients, without observing any toxicity. The only complication was mild proteinuria. Systemic bevacizumab is a promising adjuvant treatment in aggressive recurrent respiratory papillomatosis in children. It is effective and well tolerated. Further studies are needed to establish the optimal dosing frequency and duration of therapy. Laryngoscope, 129:1001–1004, 2019
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