Sacrococcygeal teratoma is the most common congenital germ cell tumor . The tumor arises from embryologically multipotent cells from the Hensen node, which is located in the coccyx. Sacrococcygeal teratomas are generally benign tumors. However, malignant elements can be present, and their frequency increases with the age of the patient. This chapter discuss the clinical features, classification, diagnosis and treatment of sacrococcygeal teratoma.