Background: Congenital Diaphragmatic Hernia (CDH) is a rare developmental abnormality of the diaphragm with an incidence of approximately 2.5 cases per 10,000 births. Despite advances in treatment, mortality and long-term morbidity among survivors remain high. Case Presentation: A newborn was delivered by planned caesarean section due to prenatal diagnosis of CDH, diagnosed at 18 weeks and 5 days (Correction 3.1) during routine ultrasound. The Observed-to-Expected (O/E) Lung area-to-Head circumference Ratio (LHR) was 52%. After birth, the newborn was in respiratory distress and required mechanical invasive ventilation. Surgical repair was planned after stabilisation for the second day of life. A subcostal laparotomy was performed, and an anteromedial hernia with mostly small intestine, the left colon flexure and the spleen as content was found. The abdominal contents were reduced, and the hernia was repaired with a suture. Due to the development of a severe pulmonary hypertension, extu