BACKGROUND: Spinal cord arteriovenous malformation (AVM) associated with spinal aneurysm is not particularly rare, but cases presenting with hematomyelia are relatively rare compared to those with subarachnoid hemorrhage (SAH). We report a rare case of successfully treated spinal AVM associated with ruptured aneurysm presenting with hematomyelia. CASE DESCRIPTION: A 52-year-old male was admitted to our hospital with sudden onset of tetraplegia, respiratory disturbance, and superficial sensory disturbance. Computed tomography revealed hematomyelia at the level of C3-4. Gadolinium-enhanced magnetic resonance imaging showed small, enhanced lesions. Angiography revealed an intradural perimedullary arteriovenous malformation associated with two aneurysms on the feeding arteries. Administration of high-dose methylprednisolone gradually ameliorated his symptoms. Direct surgical obliteration was performed on the 30th day after the onset. The bilateral C3 cervical radicular arteries and the nidus were coagulated. Angiography performed after surgery showed neither the aneurysms nor the nidus. He was discharged with only mild weakness in the left upper extremity and mild left hypesthesia 3 months after surgery, and was fully independent. CONCLUSION: We report a case of hematomyelia caused by ruptured aneurysm associated with spinal arteriovenous malformation that was successfully treated with surgical obliteration.