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Unilateral absence of a pulmonary artery is an uncommon condition and usually complicated by a cardiac anomaly. Our case is a rare one who showed the absence of the left pulmonary artery with left aortic arch and without cardiac anomaly. He suffered from recurrent hemoptysis and pneumonia since he was 9 months old. Angiography revealed peribronchial arteriovenous malformation of the affected lung which was supplied from subclavicular arteries and bronchial arteries. Although he was treated by operative ligation and angiographic embolotherapy of the supplying systemic arteries, the repeated the attacks of massive hemoptysis and necessiated left pneumonectomy at 10 years of age.
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