Background: Noninvasive monitoring of early abnormalities and therapeutic intervention in cystic fibrosis (CF) lung disease using MRI is important. Lung T 1 mapping has shown potential for local functional imaging without contrast material. Recently, it was discovered that observed lung T 1 depends on the measurement echo time (TE). Purpose: To examine TE-dependence of observed T 1 in patients with CF and its correlation with clinical metrics. Study Type: Prospective. Population: In all, 75 pediatric patients with CF (8.6 AE 6.1 years, range 0.1-23 years), with 32 reexamined after 1 year. Field Strength/Sequence: Patients were examined at 1.5T using an established MRI protocol and a multiecho inversion recovery 2D ultrashort echo time (UTE) sequence for T 1 (TE) mapping at five TEs including TE 1 = 70 μs. Assessment: Morphological and perfusion MRI were assessed by a radiologist (M.W.) with 11 years of experience using an established CF-MRI scoring system. T 1 (TE) was quantified automatically. Clinical data including spirometry (FEV1pred%) and lung clearance index (LCI) were collected. Statistical Tests: T 1 (TE) was correlated with the CF-MRI score, clinical data, and LCI. Results: T 1 (TE) showed a different curvature in CF than in healthy adults: T 1 at TE 1 was shorter in CF (1157 ms AE 73 ms vs. 1047 ms AE 70 ms, P < 0.001), but longer at TE 3 (1214 ms AE 72 ms vs. 1314 ms AE 68 ms, P < 0.001) and later TEs. The correlations of T 1 (TE) with patient age (ρ TE1-TE5 = −0.55, −0.44, −0.24, −0.30, −0.22), and LCI (ρ TE1-TE5 = −0.43, −0.42, −0.33, 0.27, −0.22) were moderate at ultra-short to short TE (P < 0.001) but decreased for longer TE. Moderate but similar correlations at all TE were found with MRI perfusion score (ρ TE1-TE5 = −0.43, −0.51, −0.47, −0.46, −0.44) and FEV1pred% (ρ TE1-TE5 = +0.44, +0.44, +0.43, +0.40, +0.39) (P < 0.05). Data Conclusion: TE should be considered when measuring lung T 1 , since observed differences between CF and healthy subjects strongly depend on TE. The different variation of correlation coefficients with TE for structural vs. functional metrics implies that TE-dependence holds additional information which may help to discern effects of tissue structural abnormalities and abnormal perfusion. Level of Evidence: 2 Technical Efficacy Stage: 1