Cardiac tamponade in systemic sclerosis is rare. We report four cases of SSc with hemodynamically significant pericardial effusions associated with pulmonary arterial hypertension, three of whom died, two following pericardiocentesis. Of 26 SSc cases reported in the literature with large pericardial effusions, seven were associated with PAH. Including our series, the mortality rate is 55%.The potential contributory role of PAH in the development of pericardial effusion and the management implications are explored. In SSc patients with hemodynamically significant pericardial effusions and severe pulmonary hypertension, initial stabilization of pulmonary artery pressure and right heart function with vasoactive therapy and then cautious pericardial drainage should be considered.