Pre-capillary pulmonary hypertension was the presenting sign of a CREST syndrome in a 65 year old woman. The diagnosis of this form of scleroderma is based on the association of a number of features (calcinosis, Raynaud's phenomenon, oesophageal dyskinesia, sclerodactylia and telangectasia). Scleroderma is the systemic disease which is usually complicated by pre-capillary pulmonary hypertension. This complication is observed in about 13% of CREST syndromes, but very rarely as severe pre-capillary pulmonary hypertension. The diagnosis of pre-capillary pulmonary hypertension carries a poor prognosis with a 2 year survival rate of about 40%. Treatment is usually with calcium inhibitors but with no effect on prognosis. The use of prostacycline and its analogue, iloprost, is an interesting therapeutic strategy, currently under evaluation. Cardiopulmonary transplantation is the only treatment of very severe forms, despite the progressive character of the condition. All cases of pre-capillary pulmonary hypertension require complete aetiological investigation to exclude a systemic disease, especially a scleroderma and, above all, a CREST syndrome.