We report the case of a man of 43 years old presenting with sarcoidosis which produced pulmonary hypertension and respiratory failure without radiological evidence of interstitial fibrosis. In spite of treatment with steroids and corticosteroids (1 mg/kg) there was progression over 2 1/2 years to respiratory failure and irreversible right heart failure and the death of the patient. At autopsy the pulmonary veins were obliterated by non caseating granulomas and there were minimal fibrotic parenchymal lesions. To our knowledge this is only the second case of sarcoidosis resulting in a veno occlusive pulmonary disorder.