In patients with osteogenesis imperfecta (OI), general anesthetic management should be carefully implemented in consideration of difficult intubation and the potential risks of cervical or mandibular fracture associated with tracheal intubation, bone fracture during postural changes, and respiratory dysfunction due to thoracic deformity. To prevent temperature elevation, moreover, many reports have recommended anesthetic management using total intravenous anesthesia (TIVA) rather than inhalation anesthetics, which contribute to temperature elevation. In an 8-year-old boy with type II (fatal type) OI (height, 81 cm; body weight, 12.4 kg), we performed general TIVA with remifentanil and propofol, using a laryngeal mask airway for airway management. All possible intra- and postoperative complications were effectively prevented, and the remifentanil requirement was high, as shown by a mean dose of 0.36 microg x kg(-1) x min(-1).