Subarachnoid hemorrhage (SAH) or intraventricle hemorrhage (IVH) with negative cerebral digital subtraction angiography (DSA) results, which are due to non-cervical spinal arteriovenous lesions, are uncommon. In this article we presented three cases from our hospital and nineteen cases from prior published literature and discussed clinical features, possible mechanisms underlying the hemorrhage and therapeutic strategies for managing this unusual entity. Our analysis revealed that headache was the most common initial symptom. Almost 60% of patients had symptoms related to the spinal cord at admission. Intramedullary arteriovenous malformations (AVM) were the most common type of malformation, and the thoracic segment was the most common location of the non-cervical spinal arteriovenous lesions. More than half of the patients had additional aneurysms. Surgery was chosen as the primary treatment modality in this series. Therefore, we speculate that thoracolumbar spinal arteriovenous lesions are an unusual cause of intracranial SAH with negative cerebral DSA results. If non-cervical spinal AVMs were associated with DSA-negative SAH, the pattern of hemorrhage could be manifested as the blood in supratentorial cisterns, the fourth ventricle or no copious blood around the foramen magnum as well (somewhat paradoxically), it depends on the timing of detection and image evaluation. The formation and the rupture of associated aneurysms were the most likely immediate cause of the intracranial SAH. If non-cervical spinal AVMs were not associated with DSA-negative SAH and all cases were genuine cases of 'SAH-of-unknown origin', the spinal AVM could be considered as incidental finding. Magnetic resonance imaging (MRI) of the complete spinal neuraxis is recommended to either exclude or identify a spinal lesion in these patients. Catheter-based spinal angiography remains the gold standard for the diagnosis of spinal vascular diseases. The decision regarding a therapeutic strategy is based on the angioarchitecture and on the type of spinal arteriovenous lesions.