Tracheostomy, frequently used to ensure airway maintenance, involves complications such as bleeding and pneumothorax. Major vessel damage is also a lethal complication that must be avoided. We report tracheostomy in a case of tortuous bracheocephalic artery. A 74-year-old female with slight dyspnea due to amyotrophic lateral sclerosis (ALS) referred for tracheostomy had a pulsating mass in front of the trachea, found in by magnetic resonance (MR) imaging and MR angiography to be a tortuous bracheocephalic artery. In surgery, the displaced bracheocephalic artery was confirmed between the sternothyroid muscle and lower pole of the thyroid gland. This vessel was carefully dissected, the thyroid gland was divided at the isthmus, and the anterior wall of the trachea was fenestrated in an inverted U shape. The flap created by fenestration protected the vessel wall and was sutured to the skin so the vessel would not be exposed to the tracheostoma. A tortuous major artery is not uncommon in the neck, and care should be taken to avoid damaging such a vessel during tracheostomy.