We report a case of esophageal cancer in a patient with a racemose hemangioma of the bronchial arteries. A 76-year-old man was admitted to the hospital with unexplained weight loss. Endoscopic examination revealed enhancing vessels in the upper to middle esophageal wall and a type 3 tumor in the lower esophagus. He underwent neoadjuvant chemotherapy with cisplatin and 5-fluorouracil and underwent a lower esophageal resection through a left thoracoabdominal incision. He was free from hemoptysis and had no recurrence of esophageal cancer for 1 year following the procedure. This case is not only rare, it supports the decision to perform an operative procedure.