Objective To investigate the adult outcomes of children with juvenile-onset recurrent respiratory papillomatosis via long-term follow-up. Study Design Retrospective study. Setting Beijing Tongren Hospital. Participants The study includes 121 patients with recurrent respiratory papillomatosis. Main outcome and measure We followed up respiratory papillomatosis patients aged least 14 years and analysed their clinical features based on recurrence-free time. Results In total, 112 (92.6%) patients underwent three or more operations. The age at initial operation was 4.3 +/- 2.9 years; 47.9% (58/121) experienced recurrence and underwent surgical treatment after age 14. At follow-up, 5% (6/121) had died, 41.3% (50/121) had been recurrence-free for 5 years or more (cured group), and 53.7% (65/121) had recurrence in the past 5 years (recurrent group). The age at the last operation was 9.2 +/- 4.6 years in the cured group. The overall operation frequency was higher in the recurrence group than in the cured group (17.8 +/- 11.9 vs 8.7 +/- 6.5). Additionally, the human papillomavirus (HPV) infection and tracheal dissemination rates were higher in the recurrence group than in the cured group (90.8% [59/65] vs 54.0% [27/50] and 26.2% [17/65] vs 10% [5/50], respectively). Conclusion The mortality rate for juvenile-onset recurrent respiratory papillomatosis is 5%. Approximately 50% of children experience recurrence and require repeated operations in adulthood. No significant difference in sex, age at initial operation or adjuvant therapy between the cured and recurrent groups was observed; however, significant between-group differences were found in overall operation frequency, aggressive disease, tracheal dissemination of papilloma, and HPV infection.