A 9-year-old girl developed a progressive unsteady gait for one month. Hyperactive reflexes of the four extremities and a spasticity of the lower extremities were found. Bilateral Babinski responses were elicited. An intradural extramedullary mass of upper thoracic spinal cord was demonstrated using magnetic resonance imaging and was confirmed as an intradural extramedullary epidermoid cyst at surgery. This epidermoid cyst was from congenital origin. No congenital anomalies such as spina bifida or dermal sinus were associated. The epidemiology, clinicalfeatures, radiology, surgical treatment and pathology of this benign tumor are discussed.