Tethered cord syndrome is a kind of spinal dysraphism with low-lying conus frequently associated with intraspinal lipoma, diastematomelia or fibrous band. The clinical manifestations varies from long periods of no overt symptoms to back pain, scoliosis, progressive neurological deficit of legs and incontinence. Therefore the diagnosis requires a strong clinical suspicion and aggressive investigation. It shows various cutaneous manifestations including hyperpigmentation, skin dimple, dermal sinus or hypertrichosis. This 34-year-old female patient has had a lozenge shaped hair tuft with terminal hair on the lumbosacral area since her birth. Her MRI scan showed dermal sinus and a low-lying conus at the lower border of L5. The filum terminale was also thickened. These findings are compatible for tethered cord syndrome. (Korean J Dermatol 2004;42(6):781-783)