RESUMEN
Few reports have described the involvement of syringomyelia associated with diastematomyelia in the etiology of neurological deficits. We reported a case with syringomyelia associated with diastematomyelia. A female patient with diastematomyelia was followed up clinically over 14 years. At the age of 8, she developed clubfoot deformity with neurological deterioration. Motor function of the right peroneus demonstrated grade 2 in manual muscle tests. Continuous intracanial bony septum and double cords with independent double dura were observed at upper thoracic spine. Magnetic resonance imaging revealed a tethering of the spinal cord and syringomyelia distal to the level of diastematomyelia. Extirpation of the osseum septum and duralplasty were performed surgically. She grew without neurological deterioration during 7 years postoperatively. A long-term followed up case with syringomyelia that was possibly secondary to the tethering of the spinal cord associated with diastematomyelia, and effective treatment with extirpation of the osseum septum and duralplasty was described.