Résumé : Introduction: The use of extracorporeal CO2 removal (ECCO2R) is increasingly employed in critically ill patients. However, the clinical evidence supporting its efficacy remains currently poor. Evidence acquisition: A systematic review using MEDLINE via PubMed was performed to identify eligible studies (until 30th September 2016). The amount of CO 2 reduction, the effect on the duration of mechanical ventilation and weaning, the impact on patients' outcome and the occurrence of complications were evaluated. The quality of evidence was evaluated according to the GRADE (Grading of Recommendations Assessment, Development and Evaluation) criteria. Evidence synthesis: Six studies were included (three evaluating patients with chronic obstructive pulmonary disease [COPD]; three evaluating patients with acute respiratory distress syndrome [ARDS]), involving 279 adult patients; 142 treated with ECCO2R and 137 controls. No study on pediatric population met the inclusion criteria for analysis. The overall quality of evidence of the two randomized trials and four case-control studies varied from moderate to very low. PaCO2 was generally reduced by 25-33% within a few hours following ECCO2R initiation. One ARDS study showed a significant decrease in the duration of mechanical ventilation, although this result was only found by post-hoc analysis. The three studies on COPD demonstrated that some patients supported by ECCO2R devices could avoid endotracheal intubation, however the ICU-LOS and survival was not influenced by ECCO2R when compared to controls. Conclusions: In COPD patients, a significantly reduced need for endotracheal intubation was reported. However, the use of ECCO2R has not shown significant improvement on the outcome of critically ill patients in the reviewed studies. Therefore appropriately powered, randomized, controlled studies are urgently needed.