Sacrococcygeal teratoma (SCT) is the most common congenital tumor of the newborn. Antenatal diagnosis of SCT is usually possible by ultrasound during the second or even earlier in the first trimester. It requires close fetal surveillance due to associated significant prenatal mortality such as fetal hydrops and high-output heart failure. Complete excision of the tumor is the mainstay of treatment and chemotherapy and radiotherapy can be conducted as adjuvant in malignant cases. Long-term follow-up is essential for assessment of recurrence and functional problems. We report a case of huge sacrococcygeal immature teratoma diagnosed by prenatal ultrasound, which successfully underwent postnatal complete excision of tumor after cesarean section. Clinical considerations are discussed.