Introduction The diastematomielia is a form of occult spinal dysraphism, which can be observed between 5 and 10% of the population, and once these 80% of which had lumbar location. The term diastematomyelia is because of Olliver in 1837 and is derived from the Greek “diastema” meaning cleft and “mielia” which refers to the spinal cord. Before 1950, this rare lesion was little known and few reported cases diagnosed postmortem. In the Mayo Clinic, 1935 to 1967, only 10 cases diagnosed during life and treated surgically were presented. After the 1950s, the prognosis of these patients improved significantly with the advent of new diagnostic techniques such as myelography, computed tomography [CT], magnetic resonance imaging (MRI), ultrasound (US), electromyography (EMG), and evoked potentials somatosensory (SSEP), treatment (surgical microscope and bipolar electrocautery), and rehabilitation of motor and sphincter. The diastematomielia is three times more common in women and occurs in any period of life, with peak incidence between 4 and 6 years old. At present, most authors deny the hereditary nature and diagnosis is often performed incidentally. Clinically, the disease presents with the following three pillars: skin disorders, orthopedic deformities, or signs and symptoms of neurological dysfunction. Patient and Methods: This is a case report of a 24-year-old girl with the following diagnosis: diastematomielia, thoracolumbar scoliosis, tethered cord (2 years' post-op). The girl consulted for thoracolumbar scoliosis and low back pain. At the physical examination, we saw thoracolumbar scoliosis, middle line scar (tethered cord post-op), skin stigmatas, and nonmotor deficit. On CT scan, we could observe a bone spur dividing the spinal canal at D9 level. On MRI, we could observe that the spinal cord was divided in two at the same level. In surgery, we found the bone spur at the center of the spinal canal splitting the spinal cord in two. We drilled the bone spur from the top to the base. We had to gently separate the two halves of the spinal cord from the bone spur to reduce the possibilities of damage. The two halves of the spinal cord were covered by dura mater. The surgery concluded with minimal bleeding, no dura mater injuries, and the patient had no deficit after the surgery. Results The patient had good postoperative evolution and went home for days after surgery with no deficits. Conclusion This is the first case of diastematomielia in 15 years. The diastematomyelia is a rare dysraphism condition. We must think of every child with peripheral stigmata of skin, tiredness when walking, orthopedic deformities, urologic symptoms, and so on. Urodynamic studies should be performed even when there are no symptoms because there may be a subclinical damage. Surgery should be performed early after the diagnosis is made.