The incidence of juvenile-onset recurrent respiratory papillomatosis (JORRP) varies worldwide, and the lack of well-adopted guidelines for use in China suggests that patients with JORRP do not receive optimal care. In America, where data are available, pediatric patients undergo an average of 4 surgeries annually and a total of >40 surgeries in their lifetimes primarily due to this condition. It is widely accepted that timely diagnosis and implementation of scientifically sound and effective interventions can prevent JORRP progression and mitigate serious complications. Notably, evidence-based guidelines to coordinate care are lacking, and there is a need to standardize clinical practice to improve outcomes for patients. The International Pediatric Otolaryngology Group issued guidelines in 2020 to improve care for patients with JORRP. However, this guideline was majorly tailored to the healthcare system in Europe and America, posing a challenge to its adoption in China. To this effect, we assembled a guideline development working group to formulate guidelines for the diagnosis and treatment of JORRP tailored to the Chinese context. The working group consisting of multidisciplinary experts with experience in managing patients with JORRP undertook qualitative and quantitative studies, conducted two rounds of Delphi consensus, and carried out multiple systematic reviews/meta-analyses to provide 24 key recommendations to 12 questions of clinical interest. We anticipate that healthcare workers, including primary care physicians and specialists managing JORRP, will find the guidelines useful, and their utilization will translate to improved outcomes for patients with the disease.