Diffuse involvement of the right pulmonary artery (PA) associated with fistula between the PA and coronary artery is report-ed in a woman with Takayasu’s arteritis. Both the subclavian arteries were totally occluded and drained by the meanderinged artery arising from both common carotid arteries. Lung perfusion scan revealed perfusion defect of right lung. Two fistulas were identified. A large fistula was between the right PA and left circumflex artery. A small fistula was between the right PA and left anterior descending artery. This is a rare case of Takayasu’s arteritis presenting with a coronary – pulmonary artery fistula that is secondary to a diffuse unilateral involvement of PA.