Congenital diaphragmatic hernia is a malformation resulting from incomplete fusion of the pleuroperitoneal membrane, it occurs in around 1/5000 live newborns, it produces in the vast majority of cases severe symptoms of respiratory failure and pulmonary arterial hypertension in this age group. Treatment is based on maintaining the best respiratory conditions in the child through intensive neonatal therapy management and, once this objective is achieved, surgical correction of the anatomical defect should proceed. Unfortunately, despite the progress in respiratory management of the seriously ill newborn, mortality from this disease is reported above 75%. We present a case in which the left diaphragmatic hernia associated with pulmonary hypertension led to a fatal outcome in a newborn, despite timely and adequate interventions.