We experienced anesthesia and perioperative management for hysterectomy in a patient with acquired angioedema. Angioedema due to C1 esterase inhibitor (C1-INH) deficiency (loss or dysfunction of C1-INH) is one of the rarest diseases, and is characterized by recurrent episodes of regional hard edema and ascites induced by mechanical stimuli or mental stress. Edema spreads to the subcutaneous and submucosal layer, and laryngeal edema may cause the upper airway obstruction. Tranexamic acid and C1-INH concentrates were administered perioperatively for prophylaxis of attacks, and combined spinal and epidural anesthesia was performed for hysterectomy. We could manage perioperative care without causing edema.