Recurrent respiratory papillomatosis (RRP) is an infrequent benign neoplasm commonly involving the upper respiratory tract with laryngeal predilection. The diagnosis and treatment of this disease are challenging due to its nonspecific clinical presentations and recurrent nature. We report here a rare juvenile case of RRP arising in the trachea without laryngeal lesions. Our experiences indicate that a high awareness of RRP and proper consideration in the right clinical context are prerequisites for early diagnosis, and a combination of multiple treatment modalities should be considered as a feasible treatment regimen.