Intramedullary vascular malformations in the high cervical region are extremely rare. Two young adult patients with vascular lesions at the level C 2 and 3, and C 2-4 are reported. In one patient selective spinal angiography revealed a combined malformation with a ventral intramedullary arteriovenous angioma and a premedullary aneurysm. This malformation was approached and removed transorally after spondylectomy of C 2. In the second patient MRI and selective angiography showed an intramedullary hemangioblastoma extending from C 2-C 4. After embolization, this lesion was surgically removed via a dorsal approach. Both patients suffered from severe neurological deficits preoperatively. MRI and selective angiography showed the exact locations of the tumors within the spinal cord and indicated a transoral approach in one case and a dorsal approach in the other. After complete obliteration and removal of the lesions, the patients showed no neurological deficits and returned to their previous professions.
