A 20-year-old woman with a history of seizures presented symptoms of walking difficulties for the past six months. Clinical examination was suggestive of a craniovertebral junction anomaly. A cerebrospinal fluid study showed mild protein elevation with no evidence of an infective pathology. Craniospinal MRI revealed diffuse nodular leptomeningeal enhancement of the brain and spinal cord. Histopathological examination was suggestive of a low-grade glioma, and the patient was diagnosed with primary diffuse leptomeningeal gliomatosis. So far, the patient has survived for more than 110 months without aggressive therapy. © 2008 Elsevier Masson SAS. All rights reserved.