Anomalous retroesophageal left brachiocephalic vein is a very rare anomaly of the brachiocephalic vein. At the time of writing only one case had been reported worldwide. The purpose of this study was to report four cases of anomalous retroesophageal left brachiocephalic vein and to evaluate the utility of multidetector computed tomography (MDCT) for investigation of the anomaly. From June 2005 to May 2008, we examined 2,146 children with congenital heart disease and 4,082 children without congenital heart disease using MDCT. The patients ranged in age from 1 day to 12 years. Maximum intensity projection reconstruction was performed to show the brachiocephalic vein. A retroesophageal left brachiocephalic vein was found in 4 of the 2,146 patients (0.19%) with congenital heart disease and in 0 of the 4,082 patients without congenital heart disease. In all four patients, the condition was confirmed during surgery for the congenital heart disease. MDCT is a good modality for evaluating the anomalous retroesophageal left brachiocephalic vein. An anomalous retroesophageal left brachiocephalic vein was more common in patients with congenital heart disease.