We report a rare case of left lung cancer in a patient with a right aortic arch. A 65-year-old woman was diagnosed to have an adenocarcinoma in the left upper lobe (S3) in addition to a right aortic arch (type II), with the left subclavian artery originating from the descending aorta. Left upper lobectomy and lymph node dissection was performed by video-assisted thoracic surgery (VATS). For the mediastinal dissection, the upper mediastinal lymph nodes were easily resected after verifying the location of the arterial ligament and the recurrent laryngeal nerve (RLN). This is the first report of using VATS to remove a lung cancer from a patient with a right aortic arch.