We present the fatal outcome in a 31-year-old woman of Latin-American origin diagnosed with dermatomyositis. There were three months between death and the onset of symptoms. The initial presentation was normal dermatological symptoms to which were shortly added clinical signs of effects on the lungs, as was shown radiologically and through pulmonary function tests which were subsequently identified histologically as Hamman-Rich syndrome. The patient was treated with high doses of corticosteroids, intravenous (IV) immunoglobulin, cyclophosphamide and cyclosporin. We carried out a review of the literature on pulmonary compromise in dermatomyositis, clinical and anatomopathological forms and treatment alternatives.