Scimitar syndrome has a variable presentation depending on the age at which the diagnosis is made. We report a case of a young woman (age 18 years) with suspected right pulmonary hypoplasia in whom a scimitar syndrome was diagnosed. Using MRI morphological findings and hemodynamic significance of the syndrome were assessed. Left-to-right shunt was calculated from blood flow measurements performed in the ascending aorta, the main pulmonary artery, and the aberrant scimitar vein.