A 53-year-old woman presented with a ruptured intramedullary aneurysmal dilatation fed by the anterior spinal artery associated with an arteriovenous malformation located in the ventral cervical spinal cord. She developed tetraparesis and respiratory dysfunction. The neurological deterioration was caused by hematomyelia due to the ruptured aneurysmal dilatation and progression of edema in the upper cervical spinal cord due to venous hypertension associated with additional hematoma in the medulla oblongata. Endovascular embolization of both C-1 and C-2 radicular arteries was performed with Guglielmi detachable coils, but components fed by small branches such as the radiculo-pial artery were not obliterated. Surgery was performed for extirpation of the arteriovenous malformation and cervical intramedullary hematoma, and excision of the aneurysmal dilatation through a transcondylar approach combined with vertebral artery transposition. Postoperatively, she overcame several complications such as pneumonia and endocarditis, and had only moderate weakness of the right upper and lower limbs. This case indicates that surgical intervention for high cervical intramedullary lesion may be very effective.