We conducted a systematic review of the use of telehealth in people with Cystic Fibrosis (CF). The studies reviewed were of adults and children with CF, and incorporated telehealth for monitoring symptoms, assessing adherence to prescribed therapies or providing a therapeutic intervention. Searches of four electronic databases returned 293 references. Eight studies met the inclusion criteria. Variability in study design and outcome measures precluded meta-analysis. Seven studies assessed telemonitoring feasibility for patient usability and acceptance, or for physiological monitoring. Two studies were randomised controlled trials, although only one showed differences in outcome between the intervention and usual care with improved spirometry stability and significantly increased antibiotic use in the intervention group. In four studies participants were asked to transmit data on spirometry (FEV(1)) or symptoms. Participant non-compliance with data reporting ranged from 43-63%. Generally, participants reported being able to use the required technology. There is insufficient evidence to reach a firm conclusion about the benefits of telehealth in people with CF, but it remains a promising area for future investigation.