A 52 year old woman with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal hypomotility and telangiectasia) and visceral involvement of the kidneys, lungs and liver is presented. The hepatic implication was a focal nodular hyperplasia of the liver which is a component not frequently recognized. Another 4 cases of this syndrome, associated to different forms of scleroderma, were described previously. Because of the way in which this case developed, this disease should be suspected when alkaline phosphatase and gamma-glutamyltranspeptidase levels are elevated. We comment on the utility of the laparoscopy and liver biopsy in diagnosing the diseased and the situation found. Finally, we discuss the pathogenic implications of the disease with scleroderma, owing to the vascular mechanism.