Background: Recurrent respiratory papillomatosis is a disease that is caused by Human papilloma virus that occurs in both children and adults. Recurrent respiratory papillomatosis tends to take a more aggressive clinical course in children than in adults. Recurrent respiratory papillomatosis can be fatal because of it is tendency to recur and spread throughout the respiratory tract. Mortality comes from more than one source: malignant transformation and respiratory tract obstruction. Case presentation: Our patient is, a female gender infant, had symptoms at a very young age and required frequent surgical debulking by micro laryngoscopy and bronchoscopy, every three to four weeks to maintain her airway. Conclusions: We present this case as first reported Saudi infant with sever extra laryngeal spread of respiratory papilloma, to highlight the challenges in the management of recurrent respiratory papillomatosis with extra laryngeal spread of respiratory papillomas and introduction of Human papilloma virus vaccine may have a positive impact on this disease.