Background:
Intramedullary spinal hemangioblastomas are known to be accompanied by syringomyelia.
Case description:
Here, we report a patient who presented with symptoms of a Chiari malformation but was found to have a D4 intramedullary hemangioblastoma with a holocord syrinx.
Conclusions:
Although rare, neurosurgeons should keep in mind the possibility of an intramedullary hemangioblastoma in patients presenting with symptoms of a Chiari malformation.