Introduction: Sacrococcygeal teratomas with intraspinal involvement are rare. To our knowledge, there is only one reported case specifically of a type-IV sacrococcygeal teratoma with intraspinal extension. Case presentation: A 1-day-old female prenatally diagnosed with a type-IV sacrococcygeal teratoma by ultrasound at 37 weeks of gestation was delivered vaginally without complications at 38 weeks and 1 day. Her weight was 3580 g. Physical examination revealed no visible or palpable tumors and no skin abnormalities. The neurological assessment was normal. The patient voided spontaneously and had a spontaneous bowel movement. Postnatal computed tomography (CT) showed a cystic mass in the pelvis with a maximum diameter of 9 cm, compressing the vagina and uterus, which resulted in hydrocolpos. The mass extended through a widened sacral canal up to the S-1 level. The nature of the connection between the mass and the thecal sac and intradural neural elements was not clear. The serum alpha-fetoprotein (AFP) level was 29,448 ng/ml, which is considered normal for the patient's age. After a multidisciplinary discussion the decision was made to proceed with a combined surgical resection. In supine position and through a laparotomy, the pelvic component was completely freed from all surrounding structures. The patient was then placed in prone position, and through a sacral laminectomy up to L5 we removed the intrasacral extension of the cystic mass. The thecal sac remained intact. The last segment of the sacrum and the coccyx were excised. The tumor was removed with en-bloc. Last, she was placed again in supine position for the closure of the abdominal incision. She recovered well from the operation. At 12-months of follow-up her development is appropriate for age, and she has no neurological deficits. Conclusion: A combined anterior-posterior-anterior surgical approach is a suitable strategy for the complete resection of type-IV SCTs that have an intraspinal extension.