Congenital diaphragmatic hernias are a rare developmental defect. Pulmonary complications are more frequently seen in right sided defects (Partridge et al., 2016). Hepatopulmonary fusion is a rare and highly mortal malformation exclusively seen in right sided congenital diaphragmatic hernias marked by the fibrovascular fusion of the liver and lung.A newborn male presented with respiratory distress and a 1-minute APGAR score of 7. A chest radiograph showed complete opacification of the right hemithorax, and a CT-Scan confirmed a congenital diaphragmatic hernia, an intrathoracic location of the liver and secondary pulmonary hypoplasia. 48 h after, intraoperative findings showed fusion of diaphragm, lung and liver tissue. Four months after, complete tissue division of the lower lobe from the fused liver segments VII/VIII and correction of the hernia defect was achieved. The patient was discharged from the hospital six months after.Partial division of tissues is described as the safest and most successful approach to hepatopulmonary fusion. The tally of all cases reported worldwide until 2020 shows higher survival rates with complete division of tissues (Ferguson DM; Congenital Diaphragmatic Hernia Study Group, 2020) Reported cases lean towards one-session surgical interventions. A two-stage surgical approach allows an initial low surgical trauma to manage compressive effects on intrathoracic structures by herniary contents and a second time for tissue division in a non-critical patient, in this case leading to long-term survival.Hepatopulmonary fusion is a rare and highly lethal malformation with scarce information available. Future multicenter studies should compare different therapeutic options and search for outcomes including but not limited to mortality.