Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by interstitial pneumonia that shows a rapid progressive course associated with a poor prognosis. We describe a 70-year-old man who presented rapidly progressive interstitial pneumonia associated with clinically ADM (C-ADM); he was successfully treated with polymyxin B-immobilized fiber column (PMX) hemoperfusion.