We report a patient diagnosed with Whipple’s disease who presented only with neurological symptoms including memory loss and confusion, seizures, abnormal ocular movements and a pyramidal syndrome. Neuro-imaging (CT and MRI) showed masses located in several areas of the brain which enhanced after gadolinium. There was also severe ependymitis, and a mass in the cervical cord. PCR in CSF was negative and the diagnosis was confirmed with electron microscopy. The patient had a dramatic response to an appropriate course of antibiotics.