An estimated 276,000 babies die within 4 weeks of birth every year, worldwide, from congenital anomalies. Better quality ultrasound screening can increase the detection of these fetal malformations in the prenatal period. Prenatal counseling for the pregnant woman and her family, regarding the nature of the disease and prognosis is necessary. Options for management in prenatal, perinatal, intrapartum, neonatal, and childhood periods need to be thoroughly discussed, so that the family can make an informed decision. A multidisciplinary approach is usually needed once a decision has been made to optimize fetal outcome, to plan for the timing and location as well as the mode of delivery. In most of the cases, vaginal delivery can be attempted. An elective cesarean delivery should be reserved for maternal concern of dystocia, certain fetal conditions that cesarean delivery will optimize perinatal outcome, or if the parents have a psychosocial determination to have a live-born infant.