Abstract
Multiple endocrine neoplasia type 2A (MEN2A), is associated with pheochromocytoma and medullary carcinoma of the thyroid. A surgical procedure in these patients can be complicated if they have any congenital heart disease (CHD). Nowadays, CHD patients are increasingly presenting at advanced age for non-cardiac surgeries, posing unique challenges to anesthesiologists. We hereby present a 44-year-old male with Eisenmenger syndrome (ES) and MEN2A, scheduled for bilateral adrenal excision and thyroidectomy. Patients with ES require meticulous and goal-directed management during non-cardiac surgery, depending upon pulmonary hypertension, cyanosis, and right ventricular dysfunction.