Cervicoisthmic pregnancy has a high risk of abortion or preterm delivery, and only 11 cases of live birth have been reported since 1980. In addition, almost all cases require blood transfusion and hysterectomy because of profuse bleeding after delivery of the placenta. A 39-year-old nulliparous woman who became pregnant after a fourth intracytoplasmic sperm injection was diagnosed with cervicoisthmic pregnancy on ultrasonography at 6 weeks’ gestation. A healthy neonate was delivered by cesarean section at 32 weeks, but hysterectomy and blood transfusion were required. Perinatal management is discussed.