Diastematomyeliais a congenital malformation in which a part of the spinal cord is split, usually at the level of the upper lumbar vertebra in the sagittal direction. It results from an abnormal adhesion between ectoderm and endoderm. Females are affected much more commonly than males (3:1).

There is complete or incomplete sagittal division of the spinal cord into two hemi cords due to the presence of an osseous, cartilaginous or fibrous septum in the central portion of the spinal canal.

The course of the disease is progressive as the patients may be asymptomatic to begin with and gradually progress to sensory motor disorder and loss of bowel and bladder control. With modern imaging techniques, various spinal dysraphism can be diagnosed earlier which can aid in improving quality of life of the individual. Treatment depends upon the symptoms. Regular neurological examinations may helps in early detection of progression of the disease and resection is done if required. We are presenting here a case of diastematomyelia, its imaging study and management.