OBJECT: To assess lung perfusion in young patients with cystic fibrosis (CF) using an arterial spin labeling (ASL) technique. MATERIALS AND METHODS: Perfusion imaging was performed in 5 healthy volunteers and 33 pediatric patients (13 ± 5 years) with CF using an ASL technique. Image quality was evaluated on a five-point scale (1 = excellent). Quantitative perfusion maps were calculated based on the modified Bloch equations. Perfusion differences between volunteers and CF patients and regional differences between lobes were analyzed using Student's t test. The association of perfusion values and forced expiratory volume in 1 s (FEV1) was analyzed using univariate regression analysis. RESULTS: Mean lung perfusion was 698 ± 67 ml/100g/min (range: 593-777 ml/100g/min) in volunteers and 526 ± 113 ml/100g/min (range: 346-724 ml/100g/min) in CF patients. Median image quality was 2 in volunteers and 3 in CF patients. In CF patients, significantly lower perfusion was observed in the upper lobes compared to healthy volunteers. Mean perfusion values significantly correlated with FEV1 (r = 0.84, P < 0.0001). CONCLUSION: ASL perfusion imaging provides lung perfusion assessment in young CF patients. This non-invasive functional imaging technique is worth being evaluated in the clinical monitoring of CF patients.