Objective：To investigate the value of transthoracic echocardiography (TTE) in the diagnosis and follow-up of anomalous origin of the right pulmonary artery from the ascending aorta (AORPA). Methods: The imaging data of 11 cases of AORPA diagnosed by operation or Computed Tomography Angiography (CTA) were analyzed retrospectively, and the value of TTE in the diagnosis and follow-up of AORPA was discussed. Results: 11 cases were confirmed by surgery or CTA.10 cases were diagnosed clearly while 1 case was missed before surgery, the diagnostic coincidence rate was 90.0%.All cases had a proximal origin from the ascending aorta in our series. A total of 11 patients with moderate to severe pulmonary arterial hypertension, and most of them were associated with other cardiovascular abnormalities.The surgery was performed in 10 cases with AORPA, and 1 case had not operated due to severe pulmonary hypertension. Postoperative follow-up of the 10 patients: 9 cases had smooth anastomosis,1 case failed due to the right pulmonary artery stenosis. Conclusion: TTE plays an important role in the non-invasive and accurate diagnosis for clinical diagnosis and follow-up of AORPA.