Segawa disease is an autosomal dominant trait characterized by progressive dystonia with marked diurnal fluctuation. Levodopa therapy dramatically improves the symptoms. We present the case of a pregnant woman with Segawa disease with a successful outcome. At the age of ten, bilateral clubfeet appeared as her first symptom. She was diagnosed as having Segawa disease and levodopa was administered with complete disappearance of the symptoms. She became pregnant and received levodopa throughout the pregnancy, and delivered a healthy male infant. A literature review and the present case indicated that out of nine pregnancies in six women with Segawa disease, all six pregnancies treated with levodopa monotherapy resulted in an uneventful obstetrical course and healthy infants; however, the remaining three pregnancies, two with levodopa and carbidopa and one without medication, resulted in fetal loss. Women with Segawa disease may be able to give birth to healthy infants with levodopa monotherapy.