Malignant transformation of juvenile-onset recurrent respiratory papillomatosis (RRP) is a rare event and the cases reported have been mainly observed in adults. We report the case of a 15-year-old girl with a history of severe RRP who died of a HPV 11-associated bronchopulmonary squamous cell carcinoma with pericardial invasion. HPV 11 was identified in nasopharyngeal and tracheal papillomas, as well as in the pericardial fluid. HPV 11 isolate was further analyzed by amplification and sequencing of the E1, E2, E4, E6, and E7 genes. Only one amino acid substitution in E4 due to natural polymorphism was observed. Exons 5-9 of the patient's tumor protein 53 (TP53) gene were sequenced and no mutations were identified. This observation confirms that malignant conversion of juvenile-onset RRP associated with HPV 11 to squamous cell carcinoma may arise in children. HPV 11-induced carcinogenesis needs to be further investigated.