Objective Few data are available regarding the surgical strategies for an anomalous origin of the left coronary artery (LCA) from the right pulmonary artery (RPA) with an intramural aortic course. We reviewed our experience in a case series of 10 children. Methods From 2007 to 2014, 10 patients (7 boys and 3 girls, aged 3 months to 11 years, median 21 months) underwent surgical repair. Before the operation, echocardiography showed the mean left ventricular ejection fraction 45% ± 10% and mean fractional shortening fraction 21% ± 7%. Moderate to severe mitral regurgitation was found in 4 patients and left ventricular aneurysm in 5 patients. The intramural aortic course of LCA was not diagnosed preoperatively in any of the patients. Results During the operation, the LCA orifice was seen and 2 types were identified: at the bifurcation of the main pulmonary artery and RPA in 3 patients, and more distal along the RPA from the bifurcation in 7 patients. In the first type, direct coronary button transfer was performed. In the second type, button transfer with unroofing of the intramural course was performed. Annuloplasty of the mitral valve was performed in 4 patients and the aneurysm was repaired with plication technique in 2 patients. Postoperatively, 2 patients died of cardiac failure. Others showed significantly improved left ventricular function at follow-up as compared with preoperative measures (mean ejection fraction 67% ± 6%, mean fractional shortening 32% ± 3%, P = .01 for both). Conclusions Careful attention should be paid to the extremely rare association of an aortic intramural course before and during an operation when dealing with anomalous LCA from the RPA. Surgical strategies for aortic reimplantation include coronary button transfer and unroofing of the intramural segment. The outcomes are encouraging. © 2016