A 27-year-old patient, originally from Martinique, presented with a progressive hepatic granulomatosis with hepatomegaly, splenomegaly, and non-icteric cholestasis, associated with bronchial granulomatosis lesions. The sarcoidosis regressed rapidly after high doses (60 mg/day) of prednisone. Portal hypertension developed later and provoked a severe hematemesis from rupture of esophageal varices. Signs of pulmonary arterial hypertension were then observed, and the diagnosis confirmed by pressure tests after catheterization, and angiography. A portocaval shunt caused the esophageal varices to subside, but the pulmonary arterial hypertension, resistant to corticotherapy, was rapidly fatal. In the case reported, the pulmonary arterial hypertension, independent of any parenchymatous lesion, was attributed to fibrosis of the arterial walls. The association of portal and pulmonary arterial hypertension with sarcoidosis is a very rare occurrence, and the pathogenesis of this association remains a debatable subject.