Recurrent respiratory papillomatosis (RRP) is the most common benign neoplasm of the larynx in children. Despite its benign histology, RRP has potentially morbid consequences and is often difficult to treat because of its tendency to recur and spread throughout the respiratory tract. Long neglected from an epidemiological standpoint, recent initiatives to better understand this disease process have been launched through coordination between the Centers for Disease Control and Prevention and the American Society of Pediatric Otolaryngology. In this clinical review, I discuss what we currently know regarding the etiology, epidemiology, and transmission of this disease. Clinical features including pertinent aspects of the history, physical examination, airway endoscopy, and other considerations are highlighted. A detailed description of the surgical and anesthetic management of these challenging cases is presented. Adjuvant modalities of surgical and nonsurgical treatment and their indications are discussed. Ongoing research initiatives and the Practice Guidelines of the Recurrent Respiratory Papillomatosis Task Force are also included.