Observational studies have indicated a high but heterogeneous prevalence of low bone mineral density for adult patients with cystic fibrosis. Fracture complications were also described. The objective of this study was to determine the prevalence of osteoporosis, osteopenia, and fractures among adult patients with cystic fibrosis. A systematic literature review was conducted using electronic databases. The keywords used were "cystic fibrosis [MeSH] AND bone density." Original studies were eligible if they reported the prevalence of osteoporosis and/or osteopenia and/or fractures in adult patients with cystic fibrosis. A meta-analysis of pooled proportions was performed. Heterogeneity was tested with the Cochran Q statistic, and in the case of heterogeneity a random effect model was used. Of 117 studies, 12 were selected, i.e., that represented a total of 1055 patients. Mean age ranged from 18.5 to 32 years (median: 28.2 years). Mean body mass index ranged from 19.9 to 22.4 (median: 20.7); 53.8% were men. The pooled prevalence of osteoporosis in adults with cystic fibrosis was 23.5% (95% CI, 16.6-31.0). The pooled prevalence of osteopenia was 38% (95% CI, 28.2-48.3). The pooled prevalences of radiological vertebral fractures and nonvertebral fractures were 14% (95% CI, 7.8-21.7) and 19.7% (95% CI, 6.0-38.8), respectively. In conclusion, this systematic literature review with meta-analysis emphasized the high prevalence of osteopenia and osteoporosis in young adults with cystic fibrosis. The prevalence of fracture was also high.