OBJECTIVE: Juvenile recurrent respiratory papillomatosis is a potentially fatal disease that affects the respiratory tract with a predilection for the larynx and trachea. Its incidence is estimated at 4.3 new cases per 1000 children in the United States. It is caused by the human papilloma virus, and its evolution is related to immunological factors. It should be considered in the differential diagnosis for children with a clinical hoarseness and upper airway obstruction, especially in immunocompromised patients. This paper reports a case of juvenile aggressive respiratory papillomatosis associated with immunosuppression in a patient treated at the Service of Head and Neck Surgery. CASE REPORT: A 4-year-old boy presented with a history of progressive dysphonia and dyspnea. He had undergone liver transplant for biliary atresia at 11-months of age and had since been on continued immunosuppressants. A nasal fibroendoscopic examination revealed extensive papillomatous lesions extending from the nasopharynx to the trachea. Chest computed tomography (CT) showed infiltration of the left mainstem bronchus suggesting extension of the lesions. The patient underwent direct laryngoscopy with resection of the upper airway lesions, which resulted in symptomatic improvement. During the postoperative follow-up, recurrence of the lesions was observed and ultimately required 8 surgeries before the disease was controlled. CONCLUSION: This is a case of a highly aggressive recurrent respiratory papillomatosis associated with immunosuppression that had a significant impact on the quality of life of the patient and his family. This disease must be considered early in the differential diagnosis of children with dysphonia and symptoms of airway obstruction, especially in immunosuppressed patients, to ensure timely diagnosis and treatment.