A case is reported of a 48-year-old man previously diagnosed as having mitral valvular disease, who was admitted for evaluation of chronic cor pulmonale. Seven years before admission, an intraoperative liver biopsy had shown multiple noncaseating granulomas. The further course was characterized by progressive chronic intrahepatic cholestasis and portal hypertension. Right heart catheterization revealed a mean pulmonary artery pressure of 43 mm Hg and a normal wedge pressure (5 mm Hg). A perfusion lung scan was normal. Open lung biopsy demonstrated noncaseating granulomas and extensive pulmonary fibrosis. To our knowledge, only one case of sarcoidosis with combined portal and pulmonary hypertension has previously been described.