Introduction: To our knowledge, muscular dystrophy and specifically, Duchenne muscular dystrophy, has not previously been associated with gross intrathecal abnormalities. Case report: We report a pediatric patient with Duchenne muscular dystrophy that was diagnosed a long time ago and recent onset urinary incontinence. MRI revealed a conus medullaris in a normal position with a fatty-infiltrated filum terminale. Operative intervention was performed entailing sectioning of the filum terminale. This resulted in resolution of the child's urinary incontinence. Conclusion: Although seemingly rare and perhaps associated by serendipity, new, unusual neurological deficits in a patient with muscular dystrophy, especially of an upper motor neuron nature, should warrant MRI to rule out pathology of the spinal cord such as tethering. © Springer-Verlag 2004.