Aims: This study aimed to investigate motor performance in children with cystic fibrosis (CF) and the relationship with respiratory and anthropometric outcome measures. Methods: A cross-sectional exploratory study investigated 12 children with CF, mean (SD) age 6.17 (0.67) years, using the Movement Assessment Battery for Children 2nd edition (MABC-2), spirometry, body weight, height, body mass index and age related anthropomorphic z-scores. Results: MABC-2 total scores indicated 9/12 (75%) children performed below average (<50th percentile), of which 4/12 children (33.3%) had motor delay and 2/12 (16.7%) were at risk for motor delay. The balance subscale showed the lowest scores, with 5/12 (41.7%) participants performing at or below the 5th percentile and a median (IQR) percentile score of 9.00 (5.00-62.50). A significant negative correlation was found between the manual dexterity subscale and both height and height for age z-scores (p=0.017 and p=0.019 respectively), as well as peak expiratory flow in liter (p=0.027). The balance subscale scores were positively correlated with forced expiratory volume in %predicted (p=0.048). No other significant correlations were found. Conclusion: Children with CF may be at risk for delayed motor development, particularly their balance skills. Poor lung function might affect motor development but further research is recommended.