Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic disorder with an incidence of approximately 1 in 4,000 live births [1]. Pulmonary arterial hypertension (PAH) is a rare but extremely life-threatening complication associated with NF1. Timely recognition of this unusual and severe association between NF1 and PAH is imperative in prolonging the survival in this specific patient population. We present the clinical outcomes of a 47-year old female previously diagnosed with NF1, who presented with progressively worsening dyspnea.