Summary

Hepatopulmonary fusion is an extremely rare accompaniment of right congenital diaphragmatic hernia. It is associated with abnormal systemic arterial supply and venous drainage of the right lung along with congenital heart disease. Children with this condition have a comparatively poor prognosis. We report a case of right congenital diaphragmatic hernia with hepatopulmonary fusion along with review of the literature with stress on diagnosis and management.