Pulmonary arterial hypertension (PAH) is an uncommon manifestation of systemic lupus erythematosus (SLE), affecting about 0.5% to 23.3% of the population worldwide. The causes of PAH associated with SLE are multifactorial. While it is generally associated with a full-blown picture of SLE, it may rarely be the presenting manifestation of the disease. We describe the case of a middle-aged woman who presented with features of severe PAH due to SLE. She was treated with vasodilators and immunosuppression (steroids and mycophenolate mofetil), with a partial response to treatment at six months follow-up.