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Unilateral pulmonary artery atresia (UPAA), commonly detected in childhood, can occasionally present in adulthood with typical symptoms of recurrent pulmonary infections, dyspnoea on exertion and haemoptysis. An 18-year-old girl presented with complaints of cough, dyspnoea on exertion and haemoptysis. Chest radiograph revealed dilated pulmonary trunk, signs of left sided volume loss and cavitating lesion in left lower zone. Pulmonary CT angiography found left pulmonary artery atresia. Endobronchial lung biopsy revealed granulomatous inflammation. Diagnosis of left sided UPAA with pulmonary tuberculosis was established. She responded well to the anti-tubercular therapy. This case highlights the importance of awareness about UPAA as a possible differential for exertional dyspnoea, recurrent chest infections, haemoptysis and pulmonary hypertension in adults.