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Pulmonary involvement in cryoglobulinemia usually is mild. We present a rare case of severe lung involvement in mixed cryoglobulinemia. A 73-year-old woman with cryoglobulinemic glomerulonephritis was admitted. Dyspnea appeared suddenly two days later. She received the diagnosis of acute lung injury according to the concept proposed by the American-European Consensus Conference. Management required corticosteroid pulse therapy and plasma exchange. This case is the first description of successful early and long-term management of severe lung involvement observed in cryoglobulinemia. Although she has been free of chest symptoms for seven months, further follow-up may be necessary to assess the potential danger of relapse.