Two females patients with CREST syndrome associated with cardiac involvement are reported. One of them has pericardial effusion just shown by echocardiography with patterns of cardiomyopathy of ventricular cavities, the latter as primary myocardial involvement, in the absence of pulmonary or systemic hypertension. The second patient has chronic cor pulmonale secondary to pulmonary hypertension verified by electrocardiography and echocardiography. It is emphasized the utility of the echocardiography and the importance of those anomalies that can lead to the patient's death, transforming the CREST towards a syndrome with potentially but late, severe systemic involvement.