Sustemic arterialization of the lung without sequestration is the rarest from of congenital anomalous systemic arterial supply to the lung, where an anomalous systemic artery arising from aorta supplies a normal unsequestrated segment of the lung. The non-sequestrated lung parenchyma which is supplied by an aberrant artery, has no parenchyma or bronchial abnormalities, and there is a normal connection with the bronchial trees. The symptoms of this disease varies. In most patients, it is often asymptomatic, but symptoms including dyspnea, hemoptysis, and central nervous system complications are possible. Here, we report a case of systemic arterialization of the lung without sequestration, which confirmed by angiography, with a review of the literature.