A 38-year-old Japanese male without any significant respiratory risk factors was admitted to our hospital because of an acute onset of fulminant pneumonia refractory to intravenous antibiotics administration and an unexplained creatine kinase elevation. He was intubated and under ventilator support on the second hospital day, but responded dramatically to i.v. pulse methylprednisone treatment. After exclusion of secondary etiologies including collagen diseases, we concluded this case as a probable acute (idiopathic) interstitial pneumonia, or Hamman-Rich syndrome, responding dramatically to the corticosteroid pulse administration.