The present systematic review examined the effectiveness of bilevel noninvasive positive pressure ventilation (NIPPV) in the management of chronic respiratory failure (CRF) due to severe stable chronic obstructive pulmonary disease (COPD). Randomised controlled trials (RCTs) and non-RCTs (crossover design) of adults with severe stable COPD and CRF receiving bilevel NIPPV via nasal, oronasal or total face mask were identified from electronic databases and manual screening of journals and reference lists. Respiratory function (gas exchange, lung function, ventilatory/breathing pattern, respiratory muscle function and work of breathing) and health-related outcomes (dyspnoea, functional status, exercise tolerance, health-related quality of life (HRQOL), morbidity and mortality) were assessed. In total, 15 studies met the inclusion criteria: six RCTs and nine non-RCTs. RCTs did not find improved gas exchange with bilevel NIPPV, while non-RCTs did. Lung hyperinflation and diaphragmatic work of breathing were reduced in a nonrandomised subset. HRQOL and dyspnoea, the least studied outcomes, showed improvement with bilevel NIPPV. In a subset of individuals on maximal medical treatment regimes for severe stable chronic obstructive pulmonary disease, bilevel noninvasive positive pressure ventilation may have an adjunctive role in the management of chronic respiratory failure through attenuation of compromised respiratory function and improvement in health-related outcomes.