We report a case of a 35 year-old-man with dermatomyositis associated with tracheopathia osteoplastica. The swallowing perturbation secondary to myositis and airway involvement by tracheopathia induced fatal outcome. Tracheopathia osteoplastica is a rare disease and occurs exclusively in men over the age of 50. The association of two rarest disease is not a fortuitous event. The common pathogenic factor may be, in this case, the occuptional exposure to silicon.