Background: Body mass index (BMI) is the primary accepted method to determine nutrition status in children with cystic fibrosis (CF); however, lean body mass (LBM) is more strongly associated with pulmonary function. Handgrip strength (HGS) measures muscle function and is reflective of LBM. The aims of this study were to assess if there was a relationship among HGS, nutrition status, and pulmonary function, to assess if HGS changed after hospitalization, and to assess if there was a relationship between HGS and nutrient intake. Methods: Twenty-three children with CF ages 6-18 years participated. BMI z-scores, nutrition risk scores, and pulmonary function were assessed about five months before, day 5-7 of, and about six weeks after hospitalization. HGS z-scores and arm anthropometrics were measured during and after hospitalization. Nutrient intakes were assessed during hospitalization. Results: Mean dominant HGS z-score was -1.95 ± 0.92 at hospitalization and -1.59 ± 1.06 at follow-up (p=0.007). Mean BMI z-score was -0.09 ± 0.64 at hospitalization and 0.06 ± 0.54 at follow-up (p=0.178). No significant relationship was found between HGS z-scores and BMI z-scores (p=0.892) or HGS z-scores and pulmonary function (p=0.340). Conclusions: HGS z-scores were lower than the standard even though mean BMI z-scores classified participants as normal nutrition status. Further research should be done utilizing a larger sample size in order to better examine HGS's potential as a nutrition assessment tool in this population.