Takayasu arteritis is well-known rare form of large vessel vasculitis. Pulmonary involvement is observed in 50% of cases. However, pulmonary hypertension is a rare complication of pulmonary artery stenosis. In this article we present a rare clinical case of Takayasus arteritis with typical stenosis of subclavian, carotid arteries, and involvement of pulmonary arteries with development of severe pulmonary hypertension. The article also contains discussion of current methods of diagnosis and treatment of pulmonary artery stenosis in Takayasus arteritis with special focus on effectiveness and complications of angioplasty and surgery.