Introduction: Deterioration of lung function is the main cause of mortality in cystic fibrosis (CF), so it is essential to study different related factors. Objective: To assess the association of pulmonary function with thoracoabdominal mobility and postural alignment in individuals with CF. Method: A cross-sectional study was performed in individuals with CF (8-17 years). Pulmonary function was assessed by spirometry. Thoracoabdominal mobility and postural alignment were evaluated by photogrammetry using the Postural Assessment Software (PAS/Sapo). Pearson correlation coefficient analysis was performed, and p < 0.05 was considered significant. Results: The following spirometric variables showed a decrease compared to predicted values: FEV1, FEV1/FVC, PEF and FEF25-75%. Postural assessment showed alterations in head horizontal alignment (HHA; 2.71 ± 2.23o), acromion horizontal alignment (AHA; 1.33 ± 1.35o), anterior superior iliac spine (ASIS) horizontal alignment (ASISHA; 1.11 ± 0.89o), angle between acromia and ASIS (AAASIS; 0.89 ± 0.39o), scapula horizontal asymmetry - T3 (SHAT3; 16.95 ± 12.03%), and asymmetry of the projection of the center of gravity within the base of support in the frontal (11.45 ± 8.10%) and sagittal (48.98 ± 18.55%) planes. A strong positive correlation was found between pulmonary function and thoracoabdominal mobility in the variables anteroposterior mobility of the upper chest (APMUC) and FVC (r = 0.818, p = 0.024), APMUC and FEV1 (r = 0.874, p = 0.010), and APMUC and FEF25-75% (r = 0.797, p = 0.032). A strong negative correlation was detected between FEV1/FVC and AHA (r = -0.761, p = 0.047). Conclusion: Our study showed in CF a reduction in pulmonary function, strong positive correlation between APMUC and pulmonary function, high prevalence of kyphoscoliosis and strong negative correlation between AHA and pulmonary function.