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A rare cause of early neonatal cyanosis: absent right pulmonary artery. Unilateral absent right pulmonary artery is a rare developmental anomaly that usually presents in late childhood and adolescence as recurrent respiratory tract infections, dyspnoea and haemoptysis. We report a case of a 2-day-old baby with respiratory distress and differential cyanosis. Echocardiogram showed pulmonary hypertension with absent right pulmonary artery. The findings were confirmed by CT angiogram. The baby improved with pulmonary vasodilators and antifailure medications.