The authors report a case of Currarino triad which had a congenital anorectal stenosis associated with a sacral defect and a presacral mass. A 1-year-old female presented with constipation since birth. Neurological deficits were not found on admission. She had had a diverting colostomy due to anorectal stenosis at another hospital before admission. Lumbar X-ray films showed bony defect caudal to the third sacral vertebra. Magnetic resonance image demonstrated a round cystic pelvic mass which was connected with a dural sac via anterior sacral defect. Posterior approach with Intradural removal of the presacral cystic mass was performed and followed by anoplasty by a pediatric surgeon. The cystic mass was verified histologically as mature teratoma with cystic change. Postoperatively, the urinary function and bowel movement remained intact. Currarino triad should be suspected and evaluated physically and radiographically in a case of congenital anorectal stenosis. Prompt recognition and close cooperation between pediatric surgeons and neurosurgeons is advisable to ensure adequate surgical treatment.