Abstract: Objective To improve the prenatal diagnosis accuracy of anomalous origin of one pulmonary artery branch from the aorta (AOPA) by analyzing fetal echocardiography features. Methods Fetal echocardiographic features were analyzed retrospectively in 6 cases with prenatal diagnosis of AOPA from June 2012 to October 2018, and the fetal echocardiography characteristics of AOPA were summarized. Results Among the 6 fetuses with AOPA, 5 cases were anomalous origin of right pulmonary artery from the aorta (AORPA), including 3 cases with isolated AORPA and 2 cases combined with Berry syndrome, and one case was anomalous origin of left pulmonary artery from the aorta (AOLPA) with tetralogy of Fallot (TOF) with an absent pulmonary valve. All cases had proximal form. Among the 6 fetuses, 2 cases had induced labor, including one case with TOF and one case combined with Berry syndrome. One case with Berry syndrome died of recurrent pneumonia and heart failure at 63 days after birth without surgical treatment. Other 2 cases with isolated AORPA underwent surgical repair after birth and recovered well after surgery. One case was just born and still under observation. Characteristic sonographical findings included: ① The main pulmonary artery was continuous with one pulmonary artery and there was no confluence with the other pulmonary artery. The diameter of normal pulmonary artery branch was widened. ② On the basis of three vessels and trachea view (3VT) and long axial view of left ventricle (LV), two-dimensional and color Doppler imaging (CDFI) showed a large vessel originated from the aortic arch, and the anomalous vessel arose proximally from the right or left lateral posterior aspect of the ascending aorta close to the aortic valve. ③ CDFI showed that the blood flow of the anomalous pulmonary artery branch was isolated and there was no exact connection with the main pulmonary artery. ④ Spectrum Doppler of the anomalous vessel of the lung confirmed that it was the anomalous origin of one pulmonary artery branch. ⑤ When other intracardiac abnormalities were not present, the inner diameter and proportion of the heart cavity were normal. Conclusion Proximal form of AOPA in the prenatal period can be accurately diagnosed by echocardiography. The 3VT and long axial view of LV are very important for the diagnosis of proximal form of AOPA.