[en] OBJECTIVE: We give the first account of failure of extracorporeal membrane oxygenation therapy secondary to congenital cystic malformation of the lung (CCAM) type 0. DESIGN: Case report. SETTING: Pediatric intensive care unit. PATIENT: A female neonate, appropriate for gestational age, with respiratory failure immediately after delivery. INTERVENTIONS: : Cardiopulmonary support with venoarterial extracorporeal membrane oxygenation. RESULTS: There was no improvement of pulmonary function, and the patient died. CCAM type 0 was diagnosed postmortem. CONCLUSIONS: CCAM type 0 should be considered as a rare differential diagnosis of irreversible lung pathologies leading to failure of extracorporeal membrane oxygenation therapy for neonatal respiratory failure.