Hepatic pulmonary fusion is a very rare disease; it can create a diagnostic and therapeutic dilemma.
We present the case of a female infant at a gestational age of 40 weeks. No antenatal diagnosis was performed. She had respiratory distress at birth secondary to a right congenital diaphragmatic hernia.
During surgery, a right defect of the diaphragm was found. During attempted reduction, it was noted that the severely hypoplastic right lung was fused to the dome of the liver. No distinct plane between the liver and the lung could be established with surgical dissection. The herniated liver was totally reducible with sacrifice of a small part of the inferior right lung lobe. After the follow-up of 2 years, the infant was asymptomatic with a good staturoponderal and psychomotor development.
The authors review the existing literature on this rare problem.