INTRODUCTION: Despite improvements of neonatal intensive care, mortality among patients born with congenital diaphragmatic hernia (CDH), remains high, and there is a significant late mortality in this cohort. OBJECTIVE: The aim of this study was to evaluate the causes of death among 251 consecutive CDH patients treated at our institution during the last 26years period. METHODS: Retrospective review of all causes of death between 1990 and 2015 of the CDH cohort prospectively collected in a database. RESULTS: Of the 251 CDH patients treated in our department since 1990, 49 were not alive by the end of 2015. Thirty-six patients (14%) died before discharge, and 13 (5%) after the first care event. The mean age at death was 262days (+/-653days, median 34days). Eighty six % (42 cases) of the fatalities occurred during the first year of life, more than half of the patients died before 1month of age and only 13% after the age of 1year (7 patients) (late mortality). The causes of early mortality (before 1year of age) were mainly cardio-respiratory, whereas GI complications occurred in the late mortality group. CONCLUSION: The most common cause of death among CDH patients is respiratory insufficiency and associated pulmonary hypertension, and most of the fatalities occur before 1year of age. Among older patients, gastrointestinal morbidity as cause of death is highly represented. LEVEL OF EVIDENCE: II.