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Downhill esophageal varices often develop because of venous hypertension caused by either superior vena cava obstruction or compression. We herein present a case of downhill esophageal varices caused by a giant goiter in a patient with postoperative Graves' disease. A 66-year-old man presented with an enlarged goiter. Gastrointestinal endoscopy revealed upper esophageal varices. This patient was successfully treated with repeated segmental embolization of the thyroid arteries that fed the goiter, followed by embolization of the inflow vein for downhill esophageal varices. Three years later, no re-enlargement of either the goiter or the appearance of downhill varices was observed. Segmental embolization therapy is thus considered to be a safe alternative for the treatment of downhill esophageal varices caused by giant goiter.