In this article, we describe the treatment of long standing juvenile-onset recurrent respiratory papillomatosis (JORRP) with eventual transformation to carcinoma in a patient who lived to the age of 73. Treatment modalities consisted of bronchoscopy and local excision initially. Later, YAG and CO2 laser debulking were used. Radiotherapy, chemotherapy with carboplatin (300 mg/m2) and 5-FU (600 mg/m2), oral methotrexate (5-7.5 mg/week), pegylated Interferon, indole-3-carbamide, and intralesional cidofovir were also utilized in the treatment of this patient. Except for methotrexate, each of the treatment regimens used in this patient, initially decreased growth of the papillomas and improved symptoms experienced by the patient. Interestingly, we found that this patient's long standing JORRP initially responded to a chemotherapy regimen of 4 cycles of carboplatin (300 mg/m2) and 5-FU (600 mg/m2) as well. Ultimately, the disease became resistant to all forms of treatment and progressed. The patient eventually succumbed to the disease after an approximate 77 year course.