BACKGROUND: Takayasu's arteritis is a chronic systemic inflammatory disease that usually affects the aorta, its primary branches and occasionally the pulmonary and coronary arteries. Female gender in reproductive age and Asian origin are known factors associated with higher disease prevalence. The clinical manifestations vary considerably and are typically caused by limb or organ ischemia illness and fever. The estimated incidence rate in the western world is 2.6 cases per million persons per year. Occasionally, exertional dyspnea can be the sole primary clinical manifestation of Takayasu's arteritis. CASE PRESENTATION: We report the case of a 57-year-old woman who was referred to our institution with increasing exertional dyspnea caused by pulmonary artery involvement in Takayasu's arteritis. In a review of the literature we discuss demographic data, clinical and radiographic findings and available therapeutic options. CONCLUSIONS: Dyspnea due to pulmonary artery involvement can be the initial symptom of Takayasu's arteritis. Simple clinical tests, including a complete pulse-status and blood pressure measuring at both arms can lead to the right diagnosis and should always be done beyond the auscultation of the heart and lungs in patients with dyspnea.