Abstract
Unilateral pulmonary vein atresia is an unusual congenital cardiovascular abnormality. It occurs due to failure of incorporation of pulmonary veins into the left atrium. It is usually diagnosed in childhood and diagnosis after adulthood is very rare. Herein we present a case of 21-year-old young adult with isolated unilateral pulmonary vein atresia who presented with unilateral interstitial lung disease.