Introduction: Pulmonary sequestration (PS) is characterized by a separate segment of the lung that receives anomalous vascular supply separate from the pulmonary arteries. Here we are report a 4-year-old girl with dyspnea and recurrent pneumonia who was finally diagnosed as a case of PS.Case presentationA 4-year-old girl was admitted to Imam-Reza hospital, Mashhad city, Iran, with a history of coughing and dyspnea from two years ago. On the CXR, consolidation can be seen in the left lower lobe. As there was a high likely hood of aspiration or pneumonia following her past medical history, the patient underwent bronchoscopy. More investigation with HRCT; revealed vascular anomaly. CMRI showed no cardiac abnormality. More findings showed a large collateral artery originated from left lateral side of abdominal aorta. This collateral artery went upward to the LLLL and anastomosed directly with two large posterior segmental tributary of the left lung pulmonary veins. Hyper-vascular pattern of both lungs was also observed in the MRI.ConclusionThis condition should be suspected in any infant with recurrent respiratory distress, or in anyone with recurrent pneumonia. To avoid misdiagnosis, PS should be considered in differential diagnosis in infants with chronic cough and dyspnea.