Pulmonary arterial hypertension (PAH) almost always appears late in cases of sclerosis sine scleroderma (ssSSc), a rare variant of the connective tissue disease systemic sclerosis (SSc), with multiorgan involvement in the absence of skin manifestations. Scleroderma refers to the presence of hardened, thickened skin that is a characteristic feature of SSc. The degree of skin involvement defines the classification of the sclerosis into limited or diffuse cutaneous conditions. Approximately 10% of patients with SSc do not have obvious skin manifestations. The rare form of ssSSc is characterized by the absence of skin thickening, but involves multiple organ systems, as seen in SSc. Without the distinctive skin thickening, ssSSc becomes a challenge to diagnose, which could delay the diagnosis until years after injury to the internal organs has occurred. Other possible findings in ssSSc are calcinosis, RP, esophageal dysfunction, and telangiectasias. Pulmonary involvement in ssSSc includes interstitial lung disease (ILD) and pulmonary hypertension. According to a systemic review of all published cases of ssSSc, pulmonary involvement was reported in 66% of cases, second to GI involvement (80%) but greater than cardiac involvement (25%). We are aware of only one published case of PAH as the initial presenting feature of ssSSc; that patient displayed symptoms of RP and dyspnea and had positive serologic test results for nucleolar-staining antinuclear antibody and antifibrillarin. Variable serologic test results have been reported in ssSSc, most commonly for anticentromere, as seen in our case, but also including antitopoisomerase, anti-RNA polymerase III, and anti-Th/To ribonucleoprotein. In general, antitopoisomerase antinuclear antibody, anti-RNA, and antifibrillarin are associated with diffuse cutaneous SSc. Anticentromere is usually associated with limited cutaneous SSc.