Although relatively rare, teratomas represent the most common neoplasm of the newborn period and the sacrococcygeal position is the most frequent extragonadal site. Teratomas are tumors that contain elements derived from more than one of the embryonic germ layers and generally all three. Roughly 60% of all sacrococcygeal teratomas contain only benign mature tissue while the remainder of tumors is divided between embryonic and malignant tissue. Fetal interventions including in utero tumor debulking, complete resection, or percutaneous coagulation of the main blood supply with radiofrequency ablation have been attempted under defined circumstances. Prompt recognition and complete surgical excision, regardless of tumor size or patient age is crucial to outcome. Teratomas and teratocarcinomas are congenital presacral lesions, which comprise 66% of all retrorectal tumors and cysts in adults. Diagnosis of retrorectal teratoma in adults is challenging, requiring thorough knowledge of pelvic anatomy and a high index of suspicion. Finally, occasionally teratocarcinomas are advanced and not resectable at presentation. Radiotherapy may have a role in palliation, but chemotherapy has not been found useful.