Systemic sclerosis is a chronic disorder characterized by vascular damage, immune dysregulation and fibrosis. Pulmonary manifestations of scleroderma include pulmonary hypertension with or without interstitial lung disease. Systemic sclerosis patients who develop pulmonary hypertension report dyspnea and functional limitation and have right and left ventricular dysfunction leading to accelerated mortality. Pulmonary hypertension is detected by echocardiography followed by diagnostic right heart catheterization. Treatment with calcium channel blockers, endothelin receptor antagonists, 5 phosphodiesterase inhibitor, and guanylate cyclase stimulator improves the outcome of systemic sclerosis patients with pulmonary hypertension.