Abstract Sickle cell anemia is a haematological disorder characterized by multiple vaso-occlusive complications, resulting in a reduced life expectancy. These patients are exposed to several triggering factors for sickle cell crises when they are submitted to cardiovascular surgeries with extracorporeal circulation. Therefore, meticulous care and perioperative management are required. This paper reports a successful case of combined cardiovascular surgery - aortic valve replacement and ascending aortic aneurysmectomy - with no serious post-operative complications. In this report, we emphasize the peculiarities of perioperative care in patients with sickle cell anemia.