Gliomatosis cerebri is an infrequent tumor of neuroepithelial origin presenting with deterioration of cognitive functions, behavioral and mental changes, motor weakness, headache, and seizures. Laboratory data are unconclusive. MRI appears to be the imaging modality of choice and mainly reveals a bilateral and diffuse infiltration of midline adjacent brain structures whose anatomical configuration remain intact. MRI- or CT-guided stereotactic biopsy is advised as the diagnostic procedure in suspected cases. A 9-year-old girl with diffuse cerebrospinal gliomatosis, investigated with sequential cranial CT scans, and MRI-verified spinal cord involvement is reported, and the corresponding literature is reviewed.