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Children with CF are living longer than ever before, and thus issues pertaining to quality of life rather than just longevity of life need to be addressed by the entire healthcare team. This article addressed the issues pertaining to the external support of the dysfunctional internal organs: the secondary musculoskeletal (postural) and neuromuscular control deficits that occur to the maturing child with CF. The research pointed towards starting PT interventions for these deficits during the pre-pubescent phase when postural deficits were just emerging, but a suggestion was also made to explore whether these deficits can be even more effectively monitored and treated at an earlier age. The dual relationship between the muscles used to meet the increased respiratory demands of CF and the normal postural demands of physical activities was described through a model based on a soda-pop can and pressure support. A pre-pubescent child with a typical progression of CF was presented as a case report to illustrate how a PT programme that was focused on postural deficits could be implemented and what type of outcomes might be possible. The child made significant changes within a relatively short time frame of 4 months, proposing that the musculoskeletal and neuromuscular systems may play a significant role in the medical and physical long-term outcomes of CF. For that reason, the physical as well as medical needs of the patient should be incorporated into a comprehensive multi-system approach to the disease across the lifespan.