Scimitar syndrome (pulmonary venolobar syndrome) is a rare anomaly of venous return to the heart, most commonly consisting of partial or total anomalous pulmonary venous return from the right lung. This is the report of a case of a 29-year-old woman at 31 weeks of gestation of pregnancy who was previously diagnosed with scimitar syndrome. MR angiography and PET-CT results which were obtained before pregnancy demonstrated vascular malformation in the inferior part of the right lung. No specific treatment was planned throughout the pregnancy due to the absence of any symptoms. The patient's first physical examination was unremarkable except mild hypertension. In her follow-up, severe preeclampsia was developed and the patient had undergone a cesarean section of a live birth at 34 weeks and 2 days of gestation. This is the first case of scimitar syndrome with pregnancy in which the cardiac status of the patient deteriorated coincidentally due to the development of another manifestation such as severe preeclampsia besides the syndrome itself.