BACKGROUND Dermatomyositis is an idiopathic inflammatory myopathy (IIM) that presents commonly with cutaneous, myopathic, and systemic manifestations. Cardiopulmonary involvement is usually paired with interstitial lung disease (ILD), which when severe can lead to pulmonary hypertension (PH) with heart failure, contributing to morbidity, poor quality of life, and mortality. However, dermatomyositis is rarely associated with PH in the absence of extensive ILD, and is infrequently considered in patients who present PH in the absence of significant lung findings. CASE REPORT We present the case of a 71-year-old woman who was admitted with signs and symptoms of heart failure after months of progressive dysphagia, proximal muscle weakness, and shortness of breath. She was subsequently diagnosed with dermatomyositis, as well as pre-capillary PH, through right-heart catheterization, but had no features of extensive ILD on imaging. She was treated with intravenous diuretics, intravenous methylprednisolone 1 g daily for 3 days, followed by oral prednisone 60 mg, and 2 doses of 75 g of intravenous immunoglobulins (IVIG), resulting in overall clinical improvement. Further IVIG treatment and re-evaluation of the need for vasodilator therapy was planned, but unfortunately the patient died following admission with hyperosmolar hyperglycemia and aspiration pneumonia after declining resuscitation and intubation. CONCLUSIONS PH in the absence of extensive ILD in dermatomyositis may be under-recognized. IIM evaluation should be considered in the rheumatological work-up of pre-capillary and combined pre-/post-capillary PH within the appropriate clinical context. Gaps exist in the understanding of the pathophysiology of PH in IIMs and its management, prognosis, and outcomes.