A congenital right diaphragmatic hernia was diagnosed in a full-term newborn who presented with respiratory distress at the age of 10 hours. The patient required respiratory support, and was operated on at age 6 days. During surgery, a central defect of the diaphragm was found. The herniated liver was only partially reducible because of intrathoracic adhesions. The patient died at 10 days of age as a result of persistent fetal circulation. Postmortem pathological examination demonstrated a central diaphragmatic defect and a nonseparable fibrous fusion between the liver and the lung.