Spina bifida is a term comonly used to refer to myelomeningocele, lumbosacral lipomas, split cord rnalformtion (diastematomyelia), meningoceles and some other neural tube defects. Split cord malformations with a fibrous, cartilaginous or bony band or spicule separating the spinal cord into hemicords, can occur as an isolated defect or either with a myelomeningocele or a lipomyelomeningocele. The occurrence of split cord malformations with lipomyelomeningocele is relatively rare in the pédiatrie neurosurgical practice. In this report, we present a one month old baby who had right leg monoparesis, with lipomyelomeningocele and split cord malformations with lipomyelomeningocele is relatively rare in the pédiatrie neurosurgical practice.