We report a patient with myotonic dystrophy, who underwent a transvaginal uterine myomectomy. Anaesthesia was induced and maintained with remifentanil and the target-controlled infusion of propofol titrated according to the bispectral index (BIS). A ProSeal laryngeal mask airway (PLMA) was inserted, and mechanical ventilation was performed without a muscle relaxant. The time from the end of anaesthesia to the patient-awakening was approximately 8 minutes. The rapid offset of the action of remifentanil and low-dose propofol facilitated early recovery without respiratory complications. The total intravenous anaesthesia using the BIS and the PLMA without a muscle relaxant is a useful anaesthetic technique in myotonic dystrophy patients.