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Isolated unilateral absence of a proximal pulmonary main artery is a rare congenital lesion which is often associated with other cardiovascular abnormalities and a diverse clinical presentation. It is usually diagnosed in childhood. Patients who survive into adulthood is uncommon. We report a case of 46 year old hypertensive and obese female who presented with progressive dyspnea. She had features of pulmonary hypertension. The diagnosis was confirmed by CT pulmonary angiography which showed absence of right pulmonary artery and conventional pulmonary angiography which showed ipsilateral lung receiving collaterals from Right coronary artery and its branches. The purpose of this report is to highlight the fact that UAPA, although a rare entity, should be kept in mind in patients with unexplained PAH and prolonged respiratory symptoms unresponsive to routine treatment modalities.