We describe a case of Freeman-Sheldon syndrome that presented some problems for anesthetic management. A 2-yr-old girl required orthopedic surgery for the bilateral lower extremities. Anesthesia was induced via a mask with oxygen (2 l.min-1), nitrous oxide (4 l.min-1) and sevoflurane (approximately 5%). Tracheal intubation by direct laryngoscopy was successfully achieved. Combined caudal epidural block was, however, avoided because spina bifida occulta was suspected. Spina bifida occulta was revealed postoperatively by X-ray. For anesthetic management of a patient with Freeman-Sheldon syndrome, the spine should be evaluated preoperatively when performing epidural/spinal anesthesia.