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Abstract Recurrent respiratory papillomatosis (RRP) is a rare disease characterized by recurrent papilloma along the aerodigestive tract. In this case, we describe a 16‐year‐old with longstanding laryngeal RRP secondary to vertical transmission of human papillomavirus (HPV) who presented with symptomatic pulmonary involvement and was successfully treated with systemic bevacizumab. The case describes the clinical and radiological improvement with therapy as well as the adverse effects that occurred and resolved with dose adjustments.