In this study, we describe a rare case of disseminated CNS malignancy of childhood. A seven-year-old boy with headache, vomiting and gait disturbances was diagnosed with non-obstructive hydrocephalus and papilledema. Initial MRI revealed no significant findings, however subsequent studies revealed bilateral extradural and leptomeningeal enhancement. Biopsy was eventually obtained with pathology report confirming leptomeningeal oligodendrogliomatosis. We discuss the recent description in literature of this rarely reported diagnosis. This entity primarily affects pediatric patients. It has a unique clincopathological identity, which can be easily mistaken for an inflammatory process. The diagnosis of this tumor can be complicated due to the rather nonspecific clinical presentation and findings on imaging.
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