Sacral agenesis, being a part of caudal regression syndrome, is a congenital spinal defect with an unknown etiopathogenesis, consisting of total or partial underdevelopment of sacrum and tethered cord, and can be accompanied by lumber spinal defects and neurologic problems. It is thought to be caused by a developmental defect, occurred at the early phases of gestation. Filum terminale is tethered due to adhesions and as a result of this, the genitourinary system in the first place, and then the musculoskeletal system and gastrointestinal system can also be affected particularly. Co-existing cardiac and respiratory defects can be found. The morbidity level can be determined by the level of sacrum’s agenesis and accompanying dysfunction degree of the organs and systems. In our article, we have presented the case that was brought by the family to the pediatrics clinic with the symptoms of tethered filum terminale, recurrent urinary system infection and intractable constipation.