Introduction: Posterior right diaphragmatic hernia is rare in newborn patients but when present, is accompanied by high mortality. Pulmonary hypoplasia seems to be the main cause of death but the presence of liver involvement remains one of the reasons for poor prognosis even when intrauterine surgery is performed.
Case description: In this article, we will present a rare case that was diagnosed by chance in a 65-year old adult presenting with an adenocarcinoma of the rectosigmoid junction and a right Bochdalek hernia with liver herniation and modification of the hepatic vein outflow with a natural right to left shunt.
Discussion: Diaphragmatic repair was performed on the patient with a mash and simultaneous colorectal resection. Intraoperatively, the exceptional natural modification of the hepatic outflow and alteration of the caval system was evident.
Conclusion: This case report represent an extremely rare anatomic variation and could be useful to give new important information on the evolution that occur in foetal life.
Keywords: Bochdalek; Hepatic vein; Liver herniation; Right diaphragmatic hernia.