The most common causes of hematomyelia are vascular anomalies, arteriovenous malformations, vasculitis, traumas, and conditions leading to hypocoagulation. In the presented clinical observations, hematomyelia developed against the background of anticoagulant administration and disruption of arteriovenous malformation. The disease manifests itself acutely, and the clinical picture depends on the level of spinal cord injury. Diagnosis of hematomyelia is straightforward and is based on neuroimaging by means of magnetic resonance imaging (MRI). There is no eff ective pathogenetic pharmacological therapy for hematomyelia; treatment is aimed at complications prevention. Taking in consideration the presence of a disabling neurologic impairment, this category of patients experiences an increased risk of venous thromboembolic disorders development, including fatal pulmonary embolism. In this regard, early measures are needed to prevent the development of venous thrombosis. The straightforward procedure of treatment is neurosurgical intervention in order to eliminate the source of bleeding and prevent repeated intramedullary hemorrhages. Timely diagnosis of hematomyelia followed by neurosurgical intervention leads to a favorable outcome of the disease, a decrease in mortality and disability.