An 11-month-old girl underwent a surgical operation for correction of a double-chambered right ventricle with a perimembranous ventricular septal defect and right-sided aortic arch. After midline sternotomy, a left innominate vein with a retroaortic route was identified. The patient underwent a surgical correction, and she had an uneventful course.An 11-month-old girl underwent a surgical operation for correction of a double-chambered right ventricle with a perimembranous ventricular septal defect and right-sided aortic arch. Chest X-ray was without pathological findings (Figure 1). After midline sternotomy and subsequent removal of the thymus, a left innominate vein with a retroaortic route was identified (Figure 2). The patient underwent a surgical correction, and she had an uneventful course. Diagnosis is usually made by echocardiography, computed tomography, and magnetic resonance imaging, but in several cases, diagnosis is made in the operation theater.Retroaortic left innominate vein incidence in patients with congenital heart defect was found to be 0.55%-0.57% and the associated anomalies were tetralogy of Fallot (TOF),