Inadequate bone mineral accrual during growth and accelerated bone demineralisation in adulthood are recognised as additional and serious complications for patients with cystic fibrosis (CF). However, little attention has been given to preventative strategies for this population. Inadequate bone accrual during childhood and adolescence, and premature bone loss, lead to a failure to attain an optimal peak bone mass, osteoporosis and fracture in patients with CF. Pharmaceutical treatments may assist in the improvement of bone in patients with CF, but these are usually not preferable for use in children. Evidence indicates that regular, weight-bearing exercise significantly enhances bone accrual in healthy children. This paper reviews the literature concerning the potential for weight-bearing exercise to improve bone mineral accrual in children with CF. All relevant literature since 1979 was obtained and reviewed from the Medline, PubMed, Cochrane and PEDro data base. Evidence concerning the efficacy of exercise for bone health in CF is lacking. There have been no controlled trials investigating the value of weight-bearing exercise for bone accrual in children with CF. As exercise may offer an effective and enjoyable strategy to improve the bone development in children who have CF, exercise should be a high priority for randomised controlled trials in this population.