Background: Fetal double aortic arch (DAA) malformation is a rare congenital heart disease. There is a lack of large sample on comparative study of prenatal ultrasound diagnosis with postnatal computed tomography angiography (CTA) diagnosis, surgery, and autopsy results.Methods: A retrospective cohort study was carried out on cases of fetal DAA that underwent a comprehensive ultrasound examination and were seen during a six-year period at seven tertiary referral centers.Results: A total of 36 cases out of 40 prenatally diagnosed DAA fetuses were confirmed by postnatal diagnosis (fetal autopsy, CTA, and surgery). In this cohort of 36 confirmed cases, 24 (67%) were isolated anomalies, while 12 (33%) were associated with intracardiac or extracardiac anomalies, and 2 (6%) had a 22q11.2 chromosome deletion. There were 9 cases of pregnancy termination with a fetal autopsy. Among the remaining 27 live births, 16 (59%) were asymptomatic and 11 (41%) received surgical treatment due to tracheal or esophageal compression symptoms, all with satisfactory outcomes. Prenatal echocardiography showed that DAA was mainly characterized by a bifurcation of the ascending aorta into the right and left aortic arch and the formation of a complete O-shaped vascular ring around the trachea on the three-vessel tracheal view. A variant in the aortic arch branching pattern was found for the first time. The airway obstruction, branching pattern and atretic arch of DAA were clearly shown by postnatal CTA.Conclusions: Fetal DAA has unique features on prenatal echocardiography and postnatal CTA, and steps and tips for systematic prenatal examination are required. The clinical outcomes of isolated DAA are favorable, even if surgery is performed due to symptoms. Determining whether other malformations or chromosomal anomalies exist is crucial for prognosis evaluation and prenatal decision-making.