This is the fourth recorded patient with extension of syringomyelia into the brain rostral to the mesencephalon verified at autopsy. A syrinx was demonstrated from the low thoracic segments of the spinal cord to the cervico-medullary junction, where a fibrovascular malformation and dural-arachnoid adhesions deformed the pyramids. Cavities in the spinal cord were continuous, with rostral glial-lined cavities in both corticospinal tracts through a system of sponge-like tubes. The rostral extent of these cavities on the right was the centrum semi-ovale above the neostriatum; on the left, the cavities extended to the diencephalon. A classical lateral-dorsal syringobulbia in the right medulla accompanied the syringoencephalomyelia (syringocephalus).