RESUMEN
Aim: We reported a rare case of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta associated with pulmonary atresia and right sided aortic arch diagnosed at a relatively late age. Case: 13 year-old girl presented to our pulmonology clinic with complaints of cough and dyspnea. On chest X-ray cardio thoracic ratio was increased and shadow of the arch was not seen on the left. On her echocardiography pulmonary arteries couldn't be demonstrated. Computed tomography angiography was performed to the patient. Right sided arch aorta with pulmonary atresia associated with an anomalous origin of the left pulmonary artery from the ascending aorta with a well developed collateral blood supply to the right lung and coexisting pulmonary infection was detected. She was managed medically. She is on the first year of her follow up. Her medical status is stable. Conclusion: We presented a case of relatively rarely seen anomalous origin of the left pulmonary artery from the ascending aorta with a rarely seen association of pulmonary atresia and wanted to take attention to its presentation in a late childhood.