The vascular malformations of the spine and spinal cord are rare diseases. Possible symptoms may consist in a transient neurological deficit, a progressive sensorimotor transverse lesion or an acute para- or tetraplegia. Damage to the spinal cord occurs by bleeding, space-occupying effects and venous congestion, rarely by steal effects. Classification of the true inborn malformations differentiates between arteriovenous malformations (AVMs), cavernomas and capillary teleangiectasias. The more frequent spinal dural arteriovenous fistula (SDAVF) of the elderly patient is a probably acquired lesion which is presented in a separate paper. Capillary teleangiectasias are mostly incidental findings but may cause differential diagnostic problems. Cavernomas are important causes of hemorrhage and may initially be obscured within the bleeding. MRI is the most relevant imaging procedure in the early diagnostic workup. In case of an AVM selective spinal angiography is required to define the type of the lesion and to decide about the appropriate therapy which may be endovascular-interventional, neurosurgical, combined or attentive.