A 47-year-old man with a history of industrial exposure and interstitial lung disease was admitted for acute pulmonary decompensation. Clinical course was characterized by severe dyspnea at rest, fever, hypoxemia, and elevated pulmonary arterial pressures. At autopsy, pulmonary problems were explained by a selective veno-occlusive process. Associated with pulmonary phlebitis was cerebral vasculitis and lymph node enlargement with erythrophagocytosis suggesting underlying viral infection. Pulmonary veno-occlusive disease should be considered in cases of pulmonary fibrosis, pulmonary hypertension with cor pulmonale, and pulmonary edema and congestion with normal left atrial pressures.