Objectives: This study aimed to evaluate the relationship between lung function and body composition in cystic fibrosis (CF) and examine the presence of normal-weight obesity (NWO), a high body fat percentage with a normal body mass index (BMI), in this population. Methods: In a pilot, cross-sectional study, 32 subjects with CF and a reference group of 20 adults without CF underwent body composition analysis with air displacement plethysmography. NWO was defined as a BMI <25 kg/m2 and body fat >30% (for women) or >23% (for men). Lung function in subjects with CF was determined by the percentage of predicted forced expiratory volume in 1 s (FEV1% predicted). Results: Despite lower BMI and fat-free mass index (P < 0.01), fat mass index and percent body fat did not differ between subjects with CF and the reference group. Among subjects with CF, FEV1% predicted was positively associated with fat-free mass index (β = 6.31 ± 2.93, P = 0.04) and inversely associated with fat mass index (β = -6.44 ± 2.93, P = 0.04), after adjusting for age, sex, and BMI. Ten subjects with CF (31%) had NWO, which corresponded with lower fat-free mass index and FEV1% predicted compared with overweight subjects (P = 0.006 and 0.004, respectively). Conclusions: Excess adiposity, particularly in the form of NWO, was inversely associated with lung function in CF. Larger prospective studies should be undertaken to confirm these findings and determine the long-term metabolic and clinical consequences of excess adiposity in CF. As the lifespan of individuals with CF increases, nutrition screening protocols, which primarily rely on BMI, may require reassessment.