Abstract
Most of the extragonadal teratomas are located in the sacrococygeal region. Teratoma with malignant sarcomatous differentiation is a rare form of germ cell tumor. The authors describe a 5-year-old-girl with sacrococygeal teratoma in which sarcomatous elements were observed. The patient was treated with complete surgical excision and adjuvant chemotherapy according to sarcoma protocols.