•There are four types of spinal cord arteriovenous malformations: paraspinal, epidural, dural and intradural (the latter affecting the cord, the nerve roots or the filum terminale).•The veins represent the key factors responsible for the neurological symptoms (either acute by rupture or thrombosis, or progressive by congestion or compression of the cord).•MRI and MRA are the first choice examinations to perform in order to diagnose spinal cord arteriovenous malformations.•Digital subtraction angiography is mandatory to understand the angioarchitectonics of the vascular malformation and to plan a proper therapeutic strategy (mainly endovascular).•In intradural shunts, a well-performed endovascular treatment offers long-term clinical stability even if the arteriovenous malformation is not totally cured.