BACKGROUND: The most dramatic diseases in the aorta are aortic dissection and aneurysm, which both of them are common in pregnant women with Marfan syndrome (MFS). According to recommendations in existing guidelines, pregnancy is not recommended in patients with severe dilation of the aorta and patients with MFS with aortic dilation > 45 mm should have prophylactic aortic repair before pregnancy.CASE REPORT: In this rare and unique report, we described a 34-year-old pregnant woman with marfanoid feature who had an approximate aortic root of 60 mm and severe aortic insufficiency. She denied terminating the pregnancy at her first prenatal visit and continued it until 30 weeks of gestation and the pregnancy terminated in the cardiac operating room due to multiple episodes of chest pain. No complication occurred during her close observation before surgery. The aortic repair was performed for her after the cesarean section.CONCLUSION: Pregnancy with severe aortic root dilation is high-risk for all patients; however, if it occurs, when the mother denies an abortion, inform the patient about its risk and continue the pregnancy with close observation and tight blood pressure (BP) control until the fetus becomes viable.