The heart is one of the major organs involved in scleroderma. Cardiac involvement can be manifested by myocardial disease, conduction system abnormalities, arrhythmias, or pericardial disease. Additionally, scleroderma renal crisis and pulmonary hypertension lead to significant cardiac dysfunction secondary to damage in the kidney and lung. This report summarizes the recent advances to further understand the types and mechanism of abnormalities in the heart in scleroderma. New cardiac technology shows significant frequencies of asymptomatic cardiac abnormalities. Further long-term studies are necessary to determine the outcome and the best approach to treatment of such abnormalities. Diastolic dysfunction has been carefully evaluated in scleroderma in recent years and appears to be more common than once realized. There is controversy as to whether this is a significant finding independent to other cardiopulmonary problems. More extensive evaluation of the conduction and the arrhythmia ablative therapy has helped manage these life-threatening complications.