Objectives: To assess the health- related quality- of- life (HRQOL) of children/adolescents with cystic fibrosis (CF)- and compare HRQOL in children managed by cystic fibrosis outreach service (CFOS) with those treated in a cystic fibrosis center (CFC). To compare HRQOL of children with CF in Queensland with previously published HRQOL data from the United States and examine the relationship between HRQOL scores and pulmonary function. Study design: Participants were children/adolescents with CF and their parents managed by the Royal Children’ s Hospital Queensl and at a CFC or CFOS. Two HRQOL surveys were used: PedsQLTM and Cystic Fibrosis Questionnaire (CFQ). Results: There were 91 CFC and 71 CFOS participants with similar demographics. PedsQLTM total summary score was statistically higher in CFOS, P =0.05. There was no significant difference in CFQ scores between groups. Queensland parents reported lower HRQOL for their children compared with US parents (P < 0.01) despite similar pulmonary function. Declining pulmonary function correlated with worse CFQ scores in adolescents, P < 0.05. Conclusions: Children living in regional Queensland reported as good as or slightly better HRQOL compared with children attending a CFC. Parent proxy HRQOL scores were generally low suggesting a reduced perception of HRQOL by parents for their children. Objectives: To assess the health-related quality of life (HRQOL) of children / adolescents with cystic fibrosis (CF) - and compare HRQOL in children managed by cystic fibrosis outreach service (CFOS) with those treated in a cystic fibrosis center ( CFC). To compare HRQOL of children with CF in Queensland with previously published HRQOL data from the United States and examine the relationship between HRQOL scores and pulmonary function. Study design: Participants were children / adolescents with CF and their parents managed by the Royal Children ’s Hospital Queensland and at a CFC or CFOS. Two HRQOL surveys were used: PedsQLTM and Cystic Fibrosis Questionnaire (CFQ). Results: There were 91 CFC and 71 CFOS participants with similar demographics. PedsQLTM total summary score was higher than CFOS, P = 0.05. There was no significant difference in CFQ scores between groups. Queensland parents reported lower HRQOL for their children compared with US parents (P <0.01) Monarch function. Declining pulmonary function correlated with worse CFQ scores in adolescents, P <0.05. Conclusions: Children living in regional Queensland reported as good as or slightly better HRQOL compared with children attending a CFC. Parent proxy HRQOL scores were generally low suggesting a reduced perception of HRQOL by parents for their children.