Juvenile laryngotracheobronchial papillomatosis (JLTBP) is a recurrent, prolonged disease usually confined to the upper airway. Rarely, tracheobronchial tree or lung parenchymal involvement occurs. The various therapeutic interventions are often unsuccessful once extralaryngeal involvement becomes apparent. Photodynamic therapy (PDT) has been successful in eradicating JLTBP in a few case reports. We present a case of extensive JLTBP with parenchymal involvement treated with multiple courses of PDT. We demonstrated temporary regression of endobronchial papillomas, but no change in parenchymal lesions. Recurrent endobronchial disease was most likely related to reinfection from parenchymal lesions inaccessible to PDT.