Lipomyelocele is an uncommon condition in neonates with a low prevalence of 0.3 – 0.6 per 10,000 live births. We report a case of 6-year-old girl with lipomyelocele who had a neurological deficit free for 6 years. Afterward, she presented with progressive neurological deficits. Her history was revealed that she had a typical cutaneous marker which was abandoned since birth. Finally, she received a surgical removal of a lipomatous mass and a dural reparation at the age of 7 years old. Unfortunately, her neurological deficits cannot be recovered.
