Case report: We report a 20-year-old male with Russell Silver syndrome and tethered cord syndrome. This patient presented with non-painful scoliosis. MRI demonstrated a conus medullaris at the superior aspect of the L3 vertebral body. Axial images demonstrated no fat within the filum terminale. There was a horizontal sacrum. The patient had no cutaneous stigmata over the lumbosacral spine indicative of occult spinal dysraphism. At operation, the filum terminale was found to be quite large at approximately 5.0 mm in diameter. Grossly, the filum was found to have several large vessels both anterior and posterior to it. Following transection of the filum the distal cord was noted to withdraw cephalad indicating caudal traction. Discussion: We believe this to be the first report of Russell Silver syndrome in conjunction with tethered cord syndrome. Following additional reports, Russell Silver syndrome may be added to the differential diagnoses for tethered cord syndrome. © Springer-Verlag 2004.