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Objectives Bone disease is observed in at least 11% of CF patients. The underlying pathogenesis is multifactorial, including nutritional factors but also a direct impact of the CF transmembrane conductance regulator (CFTR) dysfunction. The objective was to compare bone microstructure between pediatric CF patients and healthy volunteers. Methods This is a cross-sectional study with bone geometry, volumetric density (vBMD) and microstructure assessement by DXA and high resolution scanner (HRpQCT) at the distal radius and tibia in children with CF compared to age- and gender-matched healthy volunteers (HV). Results Thirty four CF children (15 girls, 19 boys, 15±2 yrs, centile for weight −0.5±1.6 and height −0.6±1.2, 8 with a past of fracture) were included. Main respiratory parameters were: forced expiratory volume in one second: 89±21%; forced vital capacity: 95±17% and forced expiratory flow between 25–75% of FCV: 62±31%. Height, weight and lumbar spine areal BMD were not significantly different between CF and HV (respectively: 158±14 vs. 162±11 cm, 47±12 vs. 51±12 kg and 0.830±0.147 vs. 0.819±0.175 g/cm 2 ). At the radius, bone geometry, vBMD and trabecular microstructure were not different between CF and HV. However, at the tibia, CF had smaller bone cross-sectional area than HV (619±153 vs. 696±122mm 2 p = 0.01). vBMD, cortical thickness and trabecular microstructure were not impaired at the distal tibia. Conclusion In our study, bone microstructure in children with CF was rather reassuring. Given their mild lung disease and good nutritional status, it seems unlikely that CFTR defect plays by itself a major role in the bone disease observed in patients with CF. [ABSTRACT FROM AUTHOR]