We describe an unusual case of a 48-year-old white woman diagnosed with positive antihistidyl-trna synthetase antibody polymyositis (PM) shortly after suffering from life-threatening acute respiratory distress syndrome (ARDS). In view of the fact that evaluation for infectious or noninfectious etiologies of ARDS was unrevealing, we reasoned that systemic inflammation in early subclinical PM may have precipitated alveolar-capillary membrane injury. One year after onset, PM has been maintained in remission with oral prednisone and azathioprine. This is the first report of a patient in whom ARDS was the presenting feature of anti-Jo-1 positive PM.