Aberrant right subclavian artery (ARSA) is a rare anomaly, in which the right subclavian artery arises directly from the aortic arch instead of originating from the brachiocephalic artery. This anomaly should be taken into consideration during surgical procedures around esophagus, such as esophagectomy. Any unintentional injury of this artery during surgical procedures could be extremely life threatening. A 56-year-old woman presented with dysphagia, with concurrent aberrant subclavian artery and esophageal cancer. The transhiatal esophagectomy was performed successfully since the anomaly was preoperatively diagnosed using computed tomography scan. The presence of ARSA during esophagectomy may be challenging, but if diagnosed preoperatively, the precise and diligent dissection of the retroesophageal space during esophagectomy, may prevent any injury to the aberrant artery and consequent complications.