The case of a patient, died at 48 hours after the birth, with transposition of the great arteries and pulmonary vascular disease grade 3 is described. The evaluation of VPD was carried out in several sections from each lung, stained with H&E and elastico-Van Gieson. These histologic features is strongly in opposition with previous reports, in which pulmonary vessels exhibits only grade 1 or even grade 0 (hypoplasia of the media) modifications at the birth. However, many authors underline that children of same age and associated defects had widely differing degrees of VPD. Because our histologic findings, it is advisable an open lung biopsy and the evaluation of pulmonary vascular disease to plan the type of repair also early in life.