Sacrococcygeal teratoma is a rare fetal neoplasm with an incidence of 1 in 40,000 births. Antenatal diagnosis is usually made after 22 weeks of gestation. Fetuses with this malformation are at risk of significant perinatal morbidity and mortality. Malignant components, coexisting with life-threatening anomalies, and chromosomal abnormalities are rare. Postulated causes of perinatal death include hydrops, dystocia, tumor rupture, preterm labor secondary to polyhydramnios, and anemia due either to hemorrhage or hemolysis within the tumor. Herein, we present a case of fetal sacrococcygeal teratoma diagnosed as early as 17 weeks of gestation.