A unicornuate uterus is a rare congenital uterovaginal anomaly. A unicornuate uterus is associated with infertility, cervical incompetence, and premature labor. A case of a 32-year-old null gravid female with a unicornuate uterus who had wished to bear a child for five years is reported. After undergoing insemination treatment with the husband’s semen, in vitro fertilization was performed and a twin pregnancy occurred. A successful outcome was achieved with the delivery of viable infants following intensive obstetric management.