Sacrococcygeal teratomas can have a wide variety of clinical presentations depending on their size, vascularity, and degree of mass effect upon adjacent structures. Intradural invasion of a sacrococcygeal teratoma is a rare variant that has generally been associated with a favorable neurologic outcome. In this report, we present the case of a neonate with paraplegia secondary to a large Altman type III sacrococcygeal teratoma with extension into the spinal canal. The neoplasm was completely removed using a combined anterior and posterior approach after preoperative embolization of the lateral sacral arteries. Pathology showed a mature teratoma. The patient remains paraplegic without evidence of tumor recurrence at 1 year of age.