Intramedullary spinal cord hemorrhage (hematomyelia) is an uncommon cause of myelopathy and can present in an acute, subacute, stepwise, or chronic fashion. Spinal vascular malformations such as intramedullary cavernomas and intradural arteriovenous malformations are the most common cause of atraumatic intramedullary spinal cord hemorrhage based on the existing literature. Additional considerations include warfarin or heparin anticoagulation, hereditary or acquired bleeding disorders, primary spinal cord tumors, spinal cord metastases, Gowers' intrasyringal hemorrhage, or a delayed complication of spinal radiation. Prompt diagnosis of hematomyelia first requires recognition of a myelopathy syndrome (transverse, central, anterior, posterior, or hemi-cord) often accompanied by sudden, severe back or neck pain and sometimes radicular pain. MRI with and without gadolinium is the preferred imaging modality. There are no clinical trials to guide the management of acute intramedullary spinal cord hemorrhage, and subsequent treatment is usually directed toward the underlying cause.