Aortitis is the inflammation of the wall of the aorta and can occur from an infection or autoimmune disease. Myelodysplastic syndrome (MDS) is characterised by abnormal haematopoiesis and a dysfunctional immune system. Autoimmune manifestations have been described in MDS. Here a case of a patient with aortitis and MDS is presented and discussed. All possible aetiologies were ruled out. The patient's symptoms resolved after she received steroids.