Background: This case report shows co-occurrence of diastematomyelia, tethered spinal cord, syringomyelia and filum terminale lipoma in a follow up case of meningomyelocele repair and hydrocephalus, which are rarely known to co-exist in a great frequency. Methods: We present a 5-year-old girl who, four years after meningomyelocele repair, developed bilateral lower limb weakness. Her filum terminale lipoma was sectioned, and the spinal cord was untethered. Results: She recovered well from surgery and experienced no neurological impairment. Conclusion: Thus, this case report aims to show the presence of primary and secondary neurulation defects in the same individual and the approach to its management.