Congenital right atrial aneurysm is a rare condition. Here we reported a 16-year-old male with giant right atrial aneurysm, atrial fibrillation, and atrial septal defect.Surgical resection of extensive right atrium, ASD repair, and maze procedure were performed. In the present case, we found extensive enlargement of right atrium protruding to the apex on the surface of the right ventricle. With the exist of atrial fibrillation, thrombus formation was always a lethal threat. Surgical treatment can provide excellent clinical results and further avoided life-threatening complications. K E Y W O R D S atrial aneurysm, atrial fibrillation, congenital heart disease Congenital right atrial aneurysm is a rare condition. The first case of surgical resection was performed by Bailey in 1955. Limited information was reported in the literature and less than half cases were submitted to surgical intervention. 1 Here we reported a 16-year-old male with giant right atrial aneurysm, complicated with an atrial septal defect (ASD) and atrial fibrillation. EchocardiographyF I G U R E 1 A Preoperative transthoracic echocardiography showed the giant right atrium and doppler showed a small atrial septal defect with a left-to-right shunt. B, Intraoperative transesophageal echocardiography showed the giant right atrium. C Preoperative X-ray showed cardiomegaly. D, Intraoperative image showed extensive enlargement of right atrium. E, Postoperative transthoracic echocardiography image showed normal size of right atrium. F, Postoperative X-ray showed significant shrink of silhouette revealed a giant right atrium of approximately 130*150 mm large and a 10 mm ASD (Figure 1A,B). Electrocardiogram (ECG) showed atrial fibrillation. Chest X-ray also revealed cardiomegaly with a large cardiothoracic ratio ( Figure 1C). Preoperative examinations were done, followed by the extensive surgical resection of right atrium, ASD repair, and maze procedure. Intraoperative inspection confirmed the giant right atrial aneurysm but a basically normal right atrial appendage ( Figure 1D). Extensive resection of the right atrial wall was performed and pathological examination found significant interstitial fibrosis. Postoperative echocardiography and chest X-ray confirmed the normal size of right atrium (Figure 1E,F), and ECG showed normal sinus rhythm.Most right atrial aneurysm did not result in any symptoms. 2 However, atrial fibrillation (as in our case), supraventricular arrhythmias, and intracardiac thrombus may occur. 3 Treatment of right atrial aneurysm is still controversial. Conservative treatment is always an option for asymptomatic patients. However, as surgical intervention is safe and may avoid unexpected complications, it's also encouraged due to its great long-term results, especially for patients with severe atrial dilation. In the present case, we found an extensive enlargement of right atrium, which deformedly protruded to and overlapped on the surface of right ventricle. With atrial fibrillation, thrombus formation is always a lethal threat. Sur...