Sacrococygeal teratomas are congenital tumors of newborns, originate from one or more of the three germinal layers. Most sacrococygeal teratomas are found in newborns, infants and children youger than 4 years. The prenatal ultrasound exams have increased the number of sacrococygeal teratomas presenting in fetuses. Radical resection, including the coccyx must be performed even if the primary lesion is benign in up to 70% of cases. We report a 16 days old baby girl presented with a large, type 111 sacrococygeal teratoma. The tumor was diagnosed only at 36 weeks of pregnancy. The teratoma was disected from the surrounding structures and excised en bloc with the coccyx. The postoperative recovery was uneventful. There are good functional and cosmetic results at six months follow-up.
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