Sacrococcygeal teratoma is a rare fetal neoplasm with an incidence of 1 in 40,000 births. Fetuses with this malformation are at risk of significant perinatal morbidity and mortality. Prenatal ultrasonographic diagnosis of sacrococcygeal teratomas is essential to detect various prenatal and perinatal complications as well as fetal outcome. Adverse clinical sequelae of a sacrococcygeal teratoma can be prevented by accurate prenatal assessment and appropriate obstetrical and perinatal management. Development of fetal hydrops and/or placentomegaly predicts fetal demise. Herein, we present a case of fetal sacrococcygeal teratoma diagnosed prenatally.Key Words: Sacrococcygeal teratoma, Prenatal diagnosis