Sacrococcygeal teratoma is a rare fetal disease but with high perinatal mortality due to sequestration of blood flow and consequent development of severe fetal anemia. We present the case of a 27 weeks pregnant woman referred to our service for prenatal management of a fetus with giant sacrococcygeal teratoma and severe anemia and who was subjected to intrauterine intravascular transfusion that could permit prolongation of the pregnancy and improve perinatal results.