Tracheomegaly, as characterized by a marked dilatation of the trachea, has not been reported in amyotrophic lateral sclerosis (ALS). Herein, we report a case of tracheomegaly secondary to tracheotomy tube cuff in a patient with ALS. To our knowledge, this is the first reporting of an ALS patient with tracheomegaly and with tracheomegaly being associated with tracheotomy tube cuff and home tracheotomy mechanical ventilator.