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Medically refractory chylous pleural effusion after congenital heart surgery is associated with significant morbidity and mortality, especially in infants. We reviewed our experience with diaphragmatic fenestration procedure in this group of patients.A retrospective chart review of all patients who had diaphragmatic fenestrations for chylous effusion at our institution over a 2-year period was performed.A total of 9 diaphragmatic fenestration procedures were performed in 8 patients who had failed medical management of chylous pleural effusions. All procedures except 1 were performed on the right side. The median age at time of procedure was 4.6 months (range, 3 weeks to 14 months). The average time between primary congenital cardiac surgery and fenestration was 26 days (range, 4–53 days). Three patients had single ventricle repair. Average time of chest tube removal after procedure was 4 days. Average time to extubation was 3 days. All patients but 1 were able to advance to full feedings without reaccumulation of chylous effusion within 12 days. No complications developed in the patients. Recurrent effusion over a median follow-up period of 19 months did not develop in the patients.Diaphragmatic fenestration is an effective and safe strategy for management of persistent chylous effusions after congenital cardiac surgery.