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We report herein a case of a male newborn with a sacrococcygeal fetiform teratoma (FT). The baby presented with a large coccygeal teratoma. The preoperative diagnosis of FT was made by plain radiography, ultra- sonography and magnetic resonance imaging. The baby was successfully treated by complete excision and pelvic floor reconstruction. Postoperative follow-up was uneventful until the teratoma recurred 11 months later as a malignancy. After undergoing a second operative procedure accompanied by chemotherapy, he has been doing well for 18 months.