We present a neonate with a complex congenital cardiopathy and a right-sided diaphragmatic hernia complicated with hepatopulmonary fusion. Radiography, abdominal US and multidetector CT (MDCT) demonstrated right-sided lung hypoplasia and liver herniation. In addition, MDCT angiography showed abnormal pulmonary vascular anatomy. At surgery, a right-sided diaphragmatic hernia with a partially herniated liver and hepatopulmonary fusion was confirmed. There was no aberrant systemic vascular supply towards the lower lobe, as seen in extralobar sequestration. MDCT angiography of the chest and upper abdomen with optimal enhancement and reconstruction of the pulmonary and hepatic vasculature can demonstrate associated anomalies in cases of suspected primary or secondary right lung hypoplasia.