Segawa's disease is a rare hereditary disease characterized by progressive dystonia with diurnal fluctuation. Segawa's disease is highly responsive to Levodopa and according to rare incidence, there were limited literature and knowledge on anesthesiological management of patient with Segawa's disease.This study described the experience of anesthesiological management during three operations of a Segawa's patient. The patient continuously received Levodopa until the day of operation. Total intravenous anesthesia was provided at the first operation for curettage and general anesthesia was provided in last two operations of caesarean section. There were no perioperative and postoperative complication in three anesthetic procedures. Keywords : Segawa's disease, Anesthesia, Caesarean section