Despite the increasing number of critically ill immunocompromised patients in intensive care unit, the outcome of different types of immunocompromised patients with respiratory failure managed with venovenous extracorporeal membrane oxygenation (VV ECMO) remains unclear. What is the overall mortality of immunocompromised patients with respiratory failure managed on VV ECMO compared to immunocompetent patients? Are there differences between different types of immunocompromised states? This is a systematic review and meta-analysis using MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials for studies of any design that reported outcomes of immunocompromised adult patients managed on VV ECMO for respiratory failure. A total of 13 studies were included. The pooled mortality among immunocompromised patients undergoing VV ECMO was 63% (95% confidence interval [CI]: 49–76%, I²: 94.23%), which was significantly higher than immunocompetent patients (odds ratio [OR]: 2.57, 95% CI: 1.22–5.41, p = 0.03, I²: 48.18%). Among immunocompromised subgroups, only patients with hematologic malignancy exhibited significantly higher mortality (OR: 5.78, 95% CI: 1.07–31.29, p = 0.05, I²: 0.00%). Immunocompromised patients with acute respiratory failure treated with VV ECMO were associated with higher mortality compared to immunocompetent patients. Mortality varied by underlying cause of immunosuppression, emphasizing the need for careful, individualized patient selection.