A 33-year-old female patient, with a 4-year history of hypertension plus a 3-year history of systemic lupus erythematosus, who had been taking high dosages of corticosteroids, has shown repetitive respiratory infections and congestive heart failure for the past 8 months. Angiocardiography confirmed the diagnosis of aortic insufficiency with aneurysmatic dilation of Valsalva's posterior sinus, ascending aorta of normal diameter and normal coronary arteries. Aortic dissection causing aortic insufficiency due to collapse of aortic leaflets was spotted during the surgery and was corrected by a bovine pericardial tube and suspension of aortic valve. The postoperative (PO) period was complicated by left-sided seizures followed by left hemiparesis and respiratory infection. She was discharged on the 25th PO day with mild left hemiparesis and in functional class I (NYHA), using medicines. We emphasize the need to consider the diagnosis of aortic dissection in patients with systemic lupus erythematosus and aortic insufficiency, specially in those who have a history of systemic arterial hypertension and long-term corticosteroid therapy.