Outcomes of neonates with congenital diaphragmatic hernia (CDH) have improved over the last three decades with the advent of enhanced prenatal risk stratification, early targeted resuscitation strategies, and advanced ventilation techniques and use of extracorporeal membrane oxygenation (ECMO). Despite these adjuncts, neonatal survival continues to be most contingent upon surgical repair of the diaphragmatic defect. Timing of definitive repair in patients who necessitate extracorporeal life support remains unclear, thus studies published in the last 10 years (January 1, 2012—December 31, 2022) were examined in order to glean consensus. Ten studies were selected based applicability and relevance to formulated PICO questions: six studies compare CDH repair on ECMO to post-decannulation, two studies investigate timing of exclusively on ECMO repair, while two studies assess implications of both clinical scenarios. There is moderate evidence to suggest that for neonates with CDH requiring ECMO, a targeted trial of weaning and repair after decannulation is recommended, as results in the best survival, lower bleeding complications and overall shorter ECMO duration. However, in infants who are deemed to require prolonged, ongoing ECMO support based on prenatal or early postnatal variables, early repair within <72 h decreases mortality, total ECMO duration and incidence of non-repair.