Paraneoplastic pemphigus is a distinct and rare autoimmune disease characterized by extensive and painful mucosal ulcerations and polymorphic desquamated skin lesions in the setting of an underlying neoplasm, typically of lymphoreticular origin. Thus difficulties in the management of anesthesia can be expected. A 66-years-old man was scheduled for removal of intraabdominal sarcoma associated with paraneoplastic pemphigus. Physical examination showed multiple erythematous bullae, crusts, plaques and target-like lesions on the whole body and desquamated erythematous skin lesions on the back and extremities. In the operating room, his right femoral artery was cannulated with a 20 G, 12.7 cm CVP catheter and left femoral and subclavian veins with 14 G, 20 cm CVP catheters, respectively. After application of 4 % lidocaine spray, his oropharynx and supraglottic area were evaluated under direct laryngoscopy and revealed multiple ulcerations on oral mucosa, but no distinct lesion on supraglottic area. Anesthesia was induced by rapid-sequence method with fentanyl, thiopental sodium and succinylcholine followed by endotracheal intubation. The endotracheal tube was held by a roll gauze around the neck and its cuff was minimally inflated to avoid overpressure against his tracheal wall. After the end of surgery, his oral cavity was suctioned with no remarkable bleeding, and tracheal wall including cuff-contacted area was evaluated under fiberoptic bronchoscopy, revealed intact wall without any bulla or ulceration. The patient was transferred to intensive care unit for proper postoperative management after extubation of endotracheal tube.