Knowledge of spinal cord arteriovenous malformations (AVMs) has recently been improved by studies on pathophysiology, neuroimaging, and genetic data. Nevertheless, the natural history of these lesions remains poorly understood. The authors present the case of an angiographic regression of a nidal-type spinal AVM at T-12 to L-1 in a 46-year-old woman with no risk factors. The natural course of untreated lesions is reviewed and discussed. To the best of the authors' knowledge, this is the first study that reports an angiographically proven complete spontaneous occlusion of a spinal AVM.