Takayasu's arteritis(aortoarteritis) is a chronic inflammatory disease involving the aorta, the arteries arising from the aorta, and the pulmonary arteries also may be involved. The inflammation leads to either stenosis and occlusion of the involved artery or formation of aneurysm or both. The arterial lesions can lead to secondary hypertension, retinopathy, cardiac involvement, cerebrovascular events, and premature death. The course and prognosis of patients with aortoarteritis show wide variation, and few authors have published systematic studies documenting the natural history of this disease. While the etiology of Takayasu's arteritis remains unknown, various modes of treatment including steroids, vascular surgery, and balloon angioplasty have been used for management of these patients. We experienced a case of Takayasu's arteritis with multiple occlusion of pulmonary arteries, which was confirmed by angiography and perfusion scan, so reported it with a review of literature.