BACKGROUND Review of "respiratory papillomatosis" (RRP) treatment with CO2 laser at Rigshospitalet. METHODS Retrospective study covering the period from 2000 to 2007. RESULTS A total of 138 surgical procedures was performed in 44 patients, 11 with "juvenile onset of RRP" (JORRP), 33 with "adult onset of RRP" (AORRP). The male/female ratio was 35/19. Mean age at onset was 5.5 years for JORRP and 41.6 years for AORRP. Annually, 3.4 new patients were seen. The average annual number of procedures was 0.6 for JORRP and 0.3 for AORRP, all performed in general anaesthesia, and using jet ventilation in 42%. CO2 laser was used in 94% of cases. No adjuvant treatment was used. Clinical remission was achieved after 1-2 procedures in 19/44 patients while the remaining patients recurred repeatedly and required surgery. Pulmonary papillomatosis was not observed. Voice quality was good or acceptable in patients in remission. HPV screening of 11 patients showed eight with HPV-6, two with HPV-11 and one with HPV6-11 and 62. CONCLUSIONS JORRP patients required surgery twice as often as AORRP patients partly due to the small dimensions of the juvenile larynx. Jet ventilation with a metal tube opening below the vocal cords and CO2-laser is recommended for routine use. National guidelines in RRP concerning diagnosis, treatment and follow-up are needed. RRP is a rare disease and centralisation is required to achieve experience with diagnostics and treatment.