Arteriovenous malformation of the vein of Galen (AVG) is a rare entity in t
he newborn with a high morbidity and mortality. We present two cases of fat
al AVG with persistent pulmonary hypertension of the newborn and significan
t pulmonary hypertension documented by autopsy histopathology. The pathophy
siology is reviewed and a proposed mechanism of the association between AVG
and pulmonary hypertension is discussed.