Background: Immunocompromised children are likely to develop a refractory acute respiratory distress syndrome (ARDS). The usefulness of providing extracorporeal life support (ECLS) to these patients is a subject of debate. The aim of our study was to report the outcomes and to compare factors associated with mortality between immunocompromised and non-immunocompromised children supported with veno-venous ECMO. Methods: We performed a retrospective monocentric study in the French pediatric ECMO center of Armand Trousseau Hospital, including all pediatric patients aged from 1 month to 18 years requiring ECLS for ARDS. Results: Between 2007 and 2018, one hundred and eleven (111) patients underwent ECMO for respiratory failure; among them twenty-five (25) were immunocompromised. Survival rate at 6 months after intensive care discharge was significantly lower for immunocompromised patients compared to non-immunocompromised ones (41.7% vs. 62.8%; p = 0.04). ARDS severity was similar between the 2 groups. Fungal pneumonias were reported only in immunocompromised patients (12.5% versus 0% in the control group; p = 0.001). Bleeding complications were significantly more frequent in the immunocompromised group and blood product transfusions were also more frequently required in this group. Conclusion: Six months after intensive care discharge, survival rate of immunocompromised children supported with ECMO for pediatric ARDS is lower than for nonimmunocompromised patients. But, the expectation for a favorable outcome is real and it is worth it if their condition is likely to be compatible with a good long-term quality of life.