The authors present a patient with diastematomyelia and associated congenital malformations of the spinal cord. They caused tethering of the spinal cord. Patient had cutaneous marks of malformation from birth. The congenital malformation was operated at the age 5 years. MRI and surgical findings confirm the existence of diastematomyelia together with thick tethered filum terminale and lipoma of filum terminale. On surgery the bony septum was removed, the filum terminale was resected and the lipoma was excised. Each of the anomalies caused the tethering of the spinal cord and restricted its physiological ascent. Only careful surgical revision with subsequent removal of all causes of the tethering of the spinal cord produced good result. Diastematomyelia is a congential malformation with some morphological variations and coexistence of other malformations. The presented case is a typical example of malformation associated with diastematomyelia.

        Key words: diastematomyelia - tethered spinal cord - neural tube defect - thick and tethered filum terminale - lipoma of filum terminale