Abstract The history of vascular anatomy of the spinal cord and spine began in the late 19th century. With recent advances in endovascular and surgical treatment of vascular lesions of the spinal cord, understanding the vascular anatomy of the spinal cord has become more important than ever. Catheter angiography is the gold standard for diagnosing vascular lesions of the spinal cord, and the vascular architecture of the spinal cord itself is relatively simple, a repetition of basic longitudinal and axial structures. This review discusses the vascular anatomy of the spine and spinal cord from the embryological point of view, as well as an overview of typical spinal arteriovenous shunt diseases mainly depending on their locations: paraspinal, epidural, dural, perimedullary, intramedullary, filum terminale/cauda equina, craniocervical junction, and related diseases.