Systemic arterial supply from the aorta to the lung is a rare congenital anomaly within the spectrum of bronchopulmonary sequestration according to Pryce's terminology. We describe our experience of this anomaly in an infant with congenital cardiac disease confirmed by multidetector CT scan. We found a systemic arterial supply from the aorta to the right lower lobe of lung without right lower lobar pulmonary artery and bronchopulmonary sequestration. This combination of congenital anomaly is most rare form.