We describe the morphologic variability and fetal diagnosis in pulmonary atresi with intact ventricular septum, within a chilean peadiatric cardiovacular center. An uncommon disease, that shows considerable morphologic heterogeneity, and with no local reports about this topic. We studied a consecutive series of 28 cases, in a five year period (1997-2002), at the Luis Calvo Mackenna Cardiovascular center. Morphologic feactures of each case were evaluated by direct review of the echocardiograms, angiocardiograms and from surgical reports. Among 28 infants, atresia was membranous in 82 per cent and muscular in 18 per cent. Right ventricle was bipartite in 30 per cent, unipartite in 6 per cent and tripartite in 64 per cent of cases. Coronary arterial abnormalities were identified in 36 per cent, and right ventricular dependent coronary circulation in 7,1 per cent. Median tricuspid annulus valvar size was -3. Fetal diagnosis was made in six of the 28 cases (21,4). In all of these prenatally diagnosed patients arrengements were made in order for the delivery to be fullfilled near by a cardiovascular center. This study shows data about the morphologic diversity and efforts on early fetal diagnose made in our country, in order asses this uncommon diseases