Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, is one of the leading causes of mortality in patients with scleroderma. With a prevalence of scleroderma ranging from ~70 to 240 patients/million, and a conservative estimate that about 10% of these patients develop PAH, scleroderma-related PAH (SSc-PAH) is a very frequent etiology of PAH (WHO Group I) throughout the world. However, trials indicate that SSc-PAH patients have a significantly poorer response to therapy compared to other forms of PAH such as idiopathic PAH. This perhaps relates to limited understanding of the pathogenesis of SSc-PAH, lack of adequate specific outcome measures (that factor in components of the cardiovascular response in these patients) and limited knowledge on the phenotypic and genotypic characteristics that underlie development of PAH and disease progression. This review discusses specific features of SSc-PAH and the potential reasons for poor outcomes, currently available and FDA-approved therapy for this syndrome, as well as needed future developments required to alter the overall poor prognosis in this disorder.