Background: Pulmonary rehabilitation (PR) in patients awaiting lung transplantation is crucial to ensure a transplant benefit. However, PR in patients with end-stage lung disease treated with noninvasive ventilation (NIV) remains an area of uncertainty. Objectives: The aim of the study was to assess the potential benefit of PR in patients awaiting lung transplantation treated with NIV. Methods: Patients awaiting lung transplantation who underwent comprehensive inpatient PR from 1998 to 2015 were retrospectively analyzed. Success of PR was assessed by comparing admission and discharge. Multivariate regression models were applied to assess the impact of long-term nocturnal NIV on PR success. Results: In total, 1,044 patients were included in the analysis. Thereof, 296 patients (28%) were treated with NIV. PR in patients treated with NIV resulted in a significant increase in 6-min walk distance (6MWD; from 250 ± 117 to 309 ± 116 m; p < 0.0001) and in various items of the Short Form Health Survey (SF)-36 questionnaire. The increase in 6MWD was higher in patients treated with NIV than in patients without NIV (59 ± 63 vs. 48 ± 55 m; p = 0.003). Furthermore, improvements of various lung function variables were higher in patients with NIV. Finally, multivariate generalized regression analysis revealed that NIV therapy was associated with improvement of 6MWD (p = 0.023) while controlling for various baseline characteristics. Conclusions: PR in patients with end-stage lung disease awaiting lung transplantation on nocturnal NIV is feasible and is associated with improvements of exercise capacity and quality of life. Furthermore, despite more advanced lung disease, patients treated with NIV have an increased benefit of PR compared to patients without NIV while awaiting lung transplantation.