Background Juvenile onset recurrent respiratory papillomatosis (JORRP) is a relatively rare disease. It affects the larynx in most cases. Because reports on JORRP to the lower respiratory tract (LRT) are few, we investigated clinical features of patients with a history of JORRP to analyze the risk factors of this disease. Methods Data from 208 JORRP patients admitted to Beijing Tongren Hospital from January 2008 to December 2010 were reviewed. Gender, age at onset of symptoms, age at first surgery, duration of symptoms before the first surgical procedure, the number of surgical procedures, mean interval between surgical interventions, and the number of tracheotomies in patients with and without LRT spread of JORRP were analyzed. The data from patients with and without tracheotomy were recorded and compared. Two cases of primary tracheal papillomatosis were reviewed. Results Papillomas extension down to the LRT was observed in 46 children (22.1%). Patients with LRT papillomatosis had a shorter time from the onset of the symptoms until the first surgery, required significantly more surgical procedures, and had a shorter mean surgical interval. Tracheotomy was performed in 13/162 (8.0%) children with laryngeal papillomatosis vs. 36/46 (78.3%) children with LRT papillomatosis. After tracheotomy, 36/49 (73.5%) children developed LRT papillomatosis and 10/157 (6.4%) children who did not have a tracheotomy developed LRT papillomatosis. Patients with tracheotomy required a significantly higher number of surgical procedures. The younger the patient had a tracheotomy, the longer the duration of cannulation was. Conclusions JORRP patients with LRT spread are prone to develop more aggressive disease. Tracheotomy resulted in a significant increase of LRT involvement. Chin Med J 2012;125(19):3496-3499