Whereas pulmonary fibrosis is a very frequent manifestation of scleroderma, pulmonary hypertension of the precapillary type is a much rarer occurrence in this affection. Two cases of scleroderma with subsequent development of a pulmonary hypertension are reported. Various hypotheses have been advanced to explain the origin of this disorder during the course of scleroderma. Rather than a direct consequence of the sclerodermic pulmonary fibrosis it probably arises secondary to the specific, diffuse, primary pulmonary vascular lesions.