Progressive respiratory failure is a common cause of death in patients with cystic fibrosis (CF). Although this may be related to the disease process itself, acute infectious problems may lead to respiratory failure requiring mechanical ventilation. Given the progressive nature of the disorder, some have suggested that the use of extracorporeal membrane oxygenation (ECMO) is contraindicated. The current study retrospectively reviewed the Extracorporeal Life Support Organization (ELSO) Registry to evaluate the outcomes following the use of ECMO in patients with CF. A total of 73 ECMO runs were identified in CF patients. There were 33 who received VV ECMO, 32 on VA ECMO, and 8 who received combined VA and VA ECMO. The overall survival rate for the cohort was 52% (38 of 73 patients). There was no difference in survival when comparing VA and VV ECMO. We noted an increasing trend for VV ECMO for this patient population over this time period. These data further support the need for a prospective study to evaluate outcomes following ECMO in this population with standardization of care across multiple centers.