We report a two-year-old female child with scimitar syndrome associated with absent pulmonary artery but with normal pulmonary artery pressure although several collaterals originated from the abdominal aorta to the right lung. To our knowledge, this is the fifth case with an absent pulmonary artery. Our case also had microphthalmia, an association not previously described in scimitar syndrome.

Keywords: scimitar syndrome, right pulmonary artery