We present seven cases of congenital absence of pulmonary valve observed during a 15 years period. There are five females and two males, aged from one day to six years. Three patients with a birth weight under 1.500 g died in the first 24 hours. All the patients had a systolic diastolic murmur and a single second sound. Two dimensional echocardiography was the procedure that allowed a definite diagnosis. In the valvular ring area a fixed ecogenic image was observed during the cardiac cycle. The pulmonary trunk and branches were dilated and the valvular ring was normal or small. In four patients the study was completed with catheterism and angiocardiography. Two were operated upon, with closing of the ventricular septal defect, aortic hemograft in pulmonary situation and reduction of pulmonary artery branches size.