Fetal sacrococcygeal teratoma (SCT) with hydrops fetalis is known to have a poor prognosis. We report a case of SCT with hydrops fetalis which was diagnosed antenatally at 30 weeks gestation. After delivery by cesarean section, the tumor was removed and the infant is alive one year after surgery with no sign of recurrence. However, he has profound brain damage, perhaps caused by hypoxia secondary to the perinatal hypovolemia. This case demonstrates that a 30 weeks' gestation, survival is possible in fetal cases of SCT with hydrops fetalis, and illustrates the importance of perinatal circulatory management.