Abstract
Scimitar syndrome is characterized by the anomalous connection of the right pulmonary venous return to the hepatic portion of the inferior vena cava. Its name is derived from the characteristic image observed in chest X-ray, CT scan, or during pulmonary angiography in cardiac catheterization. It is more common among females and rarely affects the left lung. The importance of knowing its symptoms and presentation allows for a high diagnostic suspicion, thus avoiding the underdiagnosis of the disease. The prognosis is generally good, and timely diagnosis can prevent the occurrence of complications such as pulmonary hypertension or portal hypertension. We present the case of an eight-year-old female patient, who was previously evaluated for episodes of lower respiratory tract infections at 18 months of age, detecting only dextroposition, without any diagnostic workup. She was then sent to our office at eight years of age, with the onset of exercise-induced dyspnea. A comprehensive workup was conducted, with a diagnosis of scimitar syndrome.