A tracheo-arterial fistula is a serious and life threatening potential complication of a tracheostomy. Since 1984, we experienced nine fatal cases of tracheo-arterial fistula among 60 Duchenne muscular dystrophy (DMD) patients who underwent a tracheostomy. Representative cases included a patient with lordosis (Case 8), in whom the fistula was located in the brachiocephalic artery close to the trachea, and another with severe scoliosis (Case 9), which caused the aorta to compress the trachea. Such anatomical changes can be the cause of a fistula between the trachea and brachiocephalic artery. The anatomical locations between the trachea and brachiocephalic artery are modified by thoracic deformities in DMD patients, and should be confirmed using computed tomography (CT) prior to a tracheostomy procedure. Further, during such a procedure, the tracheal stoma must be placed in a location clearly away from the arteries, and should be followed by regular post-operative examinations using CT and careful management to avoid a tracheo-arterial fistula.