Introduction: Scimitar syndrome is a rare congenital condition that is usually presented in two major types, the infantile type as a severe condition with a poor outcome (median age: 2 months) and the adult type with a better condition and good outcome. It also has a wide spectrum of clinical presentations, from being asymptomatic in the adult type to cardiac and respiratory failure and pulmonary hypertension in the infantile type. Case Presentation: In this case report, we present a 43-day-old female case referred to Taleghani Children Hospital, Gorgan, Iran, with respiratory symptoms. In a physical examination, the case had a grade II/VI murmur, dextrocardia, and respiratory distress. In evaluations, the patient had severe stenosis proximal to the left common carotid artery after its origin. The heart and mediastinum were displaced to the right side, and the thoracic aorta was normal in size and orientation. bovine type of supra-aortic arch branches in the left-sided aortic arch, hypoplasia of the right upper lobe with severe hypoplasia of the right lower lobe were seen as well. There was sequestration adjacent to the right lower lobe with a dual feeder artery directly from the abdominal aorta and celiac branch and dual draining veins. Partial anomalous pulmonary venous connection was detected in the neck, and mediastinal computed tomography (CT) angiography with reconstructed views. . Bronchoscopy revealed right main bronchus hypoplasia and right lower lobe hypoplasia. Then, with the help of angiography, scimitar syndrome diagnosis was confirmed for the patient. Conclusions: Although scimitar syndrome is an uncommon condition, it has serious complications and poor outcomes in some patients. It is necessary to be aware of this rare congenital condition and use helpful methods, such as CT angiography and angiography, to confirm the diagnosis.