Pulmonary hypertension is an uncommon complication of sarcoidosis and is usually related to the development of end-stage, interstitial fibrosis or honeycomb lung. We describe a patient with sarcoidosis who developed pulmonary hypertension in the absence of significant parenchymal fibrosis. The pulmonary hypertension in this case was caused by granulomatous destruction of arteries and veins, as documented by an open-lung biopsy and autopsy. Clinical features, including pulmonary function tests, are presented which suggested that end-stage interstitial lung disease was not causing the hypertension. Recognition of this complication of sarcoidosis is important if effective therapy is to be found.